JAX Mouse Strain Datasheet - 006570

STOCK Grm7^{Tg(SMN2)89Ahmb} Smn1^tm1Msd Tg(Hlxb9-GFP)1Tmj/J

Stock No:: 006570 |; SMN2 SMN- HB9-eGFP

Cryo Recovery

Overview

Also Known As: SMN2 SMN- HB9-eGFP

Mice of this strain carry a survival motor neuron 1 knock-out , a transgene consisting of the human survival motor neuron 2 (SMN2) gene and promoter, and a transgene expressing GFP in axons, dendrites and processes of spinal motor neurons of embryonic mice. Symptoms and neuropathology are similar to those of patients afflicted with type I proximal spinal muscular atrophy (SMA).

Genetic overview

Genetic Background	Generation

Grm7^{Tg(SMN2)89Ahmb}

Allele Type
Transgenic (Humanized sequence, Hypomorph, Inserted expressed sequence)

Smn1^tm1Msd

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout, Reporter)	Smn1	survival motor neuron 1

Tg(Hlxb9-GFP)1Tmj

Allele Type
Transgenic (Reporter)

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Research Applications

Neurobiology Research
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Details

Detailed Description

Similar to Stock No. 005024, mice that are homozygous for the targeted mutant Smn1 allele and carry the SMN2 transgene exhibit symptoms and neuropathology similar to patients afflicted with type I proximal spinal muscular atrophy (SMA). As an addition to Stock No. 005024, this line carries a transgene containing a Green Fluorescent Protein (GFP) under the direction of the mouse Hlxb9 promoter. Transgenic mice display distinct expression of GFP in dendrites, axons and soma of spinal motor neurons, allowing identification, isolation and purification of spinal motor neurons by FACS. GFP expression mimics endogenous HLXB9 expression pattern. Fluorescence is detected in axons, dendrites and processes of spinal motor neurons at embryonic day 9.5 to postnatal day 10 aged mice. This mutant mouse strain represents a model that may be useful for purification and in vivo tracking of spinal motor neurons. Mice homozygous for the Hlxb9-GFP transgenic insert are reportedly viable, fertile, do not display any gross behavioral abnormalities, but are smaller in size than wild-type littermates. Homozygous pups born to homozygous females have a high mortality rate. In the initial characterization by the donating investigator, mice that are homozygous for the targeted mutant Smn1 allele and carry the SMN2 transgene exhibit symptoms and mice were either stillborn or survived 4-6 days. Mice that died at or shortly after birth were slightly smaller (1.33 g. vs. 1.51 g.) than normal littermates. Mice that survive for several days are indistinguishable from normal littermates in the first 48 hours, after which they exhibit decreased suckling and movement, labored breathing and tremoring limbs. Mice succumbing at this later time point are noticeably smaller than normal littermates (1.47 g vs. 4.59). A bell-shaped trunk is also noticeable in affected mice, presumably from intercostal muscle weakness, a characteristic of type I SMA. Histological analysis indicates that affected mice that survive to day 5 exhibit a loss of motor neurons from spinal cord (35%) and facial nucleus (40%). A large number of cells with pyknotic nuclei are observed in these tissues. Immunohistochemical analysis indicates low-level expression of the SMN2 protein in the tissues examined (brain, liver, spinal cord) and an absence or near absence of intranuclear aggregates of the SMN protein (gems?). Homozygous mice bearing the Smn1 targeted mutation without a copy of the SMN2 transgene display an embryonic lethal phenotype with developmental arrest occurring prior to implantation.

Also note, Stock No. 005024 does not survive as hemizygous for the Tg(SMN2)89 transgene (SMN2+/-) and homozygous for the Smn1^tm1Msd mutation (Smn-/-).

Distribution of this model is supported by the Spinal Muscular Atrophy Foundation.

Development

The targeted mutant allele was created in the laboratory of Dr. Michael Sendtner at the University of Wurzburg, Germany. Exon 2 of the endogenous mouse Smn1 gene was disrupted by employing a targeting vector encoding a neomycin cassette and a lacZ gene fused to the first 40 nucleotides of the disrupted exon to permit expression of the lacZ gene in tissues where Smn is normally expressed. The construct was electroporated into 129P2/OlaHsd-derived E14Tg2a-IV embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts and chimeric animals obtained. Chimeric animals were crossed to C57BL/6 for an unspecified number of generations. The SMN2 transgene was created in the laboratory of Dr. Arthur Burghes at The Ohio State University. A 35.5 kb BamHI genomic fragment encoding the human SMN2 promoter and gene (derived from genomic clone PAC215P15) was injected into fertilized FVB/N mouse oocytes. Transgenic SMN2 mice from founder line 89 were established and then bred to the Smn1 mutant mice. The double mutant mice were then backcrossed to FVB/N for at least 5 generations. Next, these mice were crossed Stock No. 005029 which consisted of a transgenic construct containing a 9kb sequence of the 5' portion of the mouse Hlxb9 gene, a GFP open reading frame, and bovine growth hormome polyadenylation site sequence.

Expression Data

Expressed Gene	SMN2, survival of motor neuron 2, centromeric, human
Site of Expression	Dendrites, axons, and soma of spinal motor neurons display distinct expression of GFP. GFP expression mimics endogenous HLXB9 expression pattern. Fluorscence is detected in axons, dendrites, and processes of spinal motor neurons at embryonic day 9.5 to postnatal day 10 aged mice.
Expressed Gene	lacZ, beta-galactosidase, E. coli
Site of Expression	The expression of the lacZ gene in tissues where Smn is normally expressed was noted.
Expressed Gene	GFP, Green Fluorescent Protein,
Site of Expression	Dendrites, axons, and soma of spinal motor neurons display distinct expression of GFP. GFP expression mimics endogenous HLXB9 expression pattern. Fluorscence is detected in axons, dendrites, and processes of spinal motor neurons at embryonic day 9.5 to postnatal day 10 aged mice.

Control Suggestions

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Additional Information

Considerations for Choosing Controls

Genetics

Grm7^{Tg(SMN2)89Ahmb}

Allele Symbol: Grm7^{Tg(SMN2)89Ahmb}

Allele Name	transgene insertion 89, Arthur H M Burghes
Allele Type	Transgenic (Humanized sequence, Hypomorph, Inserted expressed sequence)
Allele Synonym(s)	SMN2
Gene Symbol and Name	Grm7, glutamate receptor, metabotropic 7
Gene Synonym(s)	6330570A01Rik; 6330570A01Rik; BB176677; E130018M02Rik; E130018M02Rik; G protein-coupled receptor, family C, group 1, member G; GLUR7; GPRC1G; Gpr1g; Gpr1g; MGLU7; MGLUR7; PPP1R87; RIKEN cDNA 6330570A01 gene; RIKEN cDNA E130018M02 gene; SMN2; Tg(SMN2)89Ahmb; Tg(SMN2)89Ahmb; expressed sequence BB176677; mGlu7a receptor; mGluR7; transgene insertion 89, Arthur H M Burghes
Promoter	SMN2, survival of motor neuron 2, centromeric, human
Expressed Gene	SMN2, survival of motor neuron 2, centromeric, human
Site of Expression	Dendrites, axons, and soma of spinal motor neurons display distinct expression of GFP. GFP expression mimics endogenous HLXB9 expression pattern. Fluorscence is detected in axons, dendrites, and processes of spinal motor neurons at embryonic day 9.5 to postnatal day 10 aged mice.
Strain of Origin	FVB/N
Chromosome	6
Molecular Note	A 35.5 kb genomic fragment containing the human survival motor neuron 2 (SMN2) gene and promoter was used for the transgene. The transgene is ubiquitously expressed in all tissues examined by Northern blot analysis. Line 89 carries 1 copy of the transgene integrated into intron 4 of the gene. RT-PCR confirmed reduced expression of the gene the transgene is integrated into.
Mutations Made By	Arthur Burghes, The Ohio State University

Smn1^tm1Msd

Allele Symbol: Smn1^tm1Msd

Allele Name	targeted mutation 1, Michael Sendtner
Allele Type	Targeted (Null/Knockout, Reporter)
Allele Synonym(s)	SMN^-
Gene Symbol and Name	Smn1, survival motor neuron 1
Gene Synonym(s)	AI849087; BCD541; C-BCD541; GEMIN1; SMA; SMA1; SMA2; SMA3; SMA4; SMA@; SMN; SMNC; SMNT; Smn; Smn; T-BCD541; TDRD16A; TDRD16B; expressed sequence AI849087; survival motor neuron
Expressed Gene	lacZ, beta-galactosidase, E. coli
Site of Expression	The expression of the lacZ gene in tissues where Smn is normally expressed was noted.
Strain of Origin	129P2/OlaHsd
Chromosome	13
Molecular Note	A lacZ-neo cassette was inserted into exon 2 by homologous recombination resulting in an in-frame fusion of lacZ to exon 2. Homozygous mutant embryos were identified up to 80 hours post coitum. The expression of the lacZ gene in tissues where Smn is normally expressed was noted.
Mutations Made By	Michael Sendtner

Tg(Hlxb9-GFP)1Tmj

Allele Symbol: Tg(Hlxb9-GFP)1Tmj

Allele Name	transgene insertion 1, Thomas M Jessell
Allele Type	Transgenic (Reporter)
Allele Synonym(s)	Gfp-HB9; Hb9-Gfp; Hb9-eGFP; Hb9::EGFP; Hb9:GFP-1B; Hlxb9:GFP
Gene Symbol and Name	Tg(Hlxb9-GFP)1Tmj, transgene insertion 1, Thomas M Jessell
Gene Synonym(s)	Gfp-HB9; Hb9-Gfp; Hb9::EGFP; Hb9:GFP-1B; Hlxb9:GFP
Promoter	Mnx1, motor neuron and pancreas homeobox 1, mouse, laboratory
Expressed Gene	GFP, Green Fluorescent Protein,
Site of Expression	Dendrites, axons, and soma of spinal motor neurons display distinct expression of GFP. GFP expression mimics endogenous HLXB9 expression pattern. Fluorscence is detected in axons, dendrites, and processes of spinal motor neurons at embryonic day 9.5 to postnatal day 10 aged mice.
Strain of Origin	(C57BL/6 x CBA)F1
Chromosome	UN
General Note	Homozygous transgenic mice on a genetic background that involves C57BL/6 and CBA are viable and fertile. They do not display any gross behavioral abnormalities, but are smaller in size than wildtype littermates. Homozygous pups born to homozygous females have a high mortality rate. Transgenic mice display distinct expression of GFP in dendrites, axons, and soma of spinal motor neurons, allowing identification, isolation and purification of spinal motor neurons by FACS. GFP expression mimics endogenous HLXB9 expression pattern. Fluorescence is detected in axons, dendrites and processes of spinal motor neurons at embryonic day 9.5 to postnatal day 10 aged mice.
Molecular Note	The transgenic construct contains a 9kb sequence of the 5' portion of the mouse Hlxb9 gene, a Green Fluorescent Protein (GFP) open reading frame, and a bovine growth hormome polyadenylation site sequence.
Mutations Made By	Ivo Lieberam, Columbia University/HHMI

Disease/Phenotype

Disease Terms

Research Areas By Genotype

This mouse can be used to support research in many areas including:

Neurobiology Research
- Fluorescent protein expression in neural tissue
- Neurodegeneration
- Spinal Muscular Atrophy (SMA)
Research Tools
- Fluorescent Proteins
- Genetics Research
  - Tissue/Cell Markers
  - Tissue/Cell Markers: neurons
- Neurobiology Research
  - cell marker

Genotype: GFP related

Research Tools
- Fluorescent Proteins

Genotype: Smn1^tm1Msd related

Neurobiology Research
- Spinal Muscular Atrophy (SMA)

References

Additional - Grm7^{Tg(SMN2)89Ahmb} related

Ahmad S; Wang Y; Shaik GM; Burghes AH; Gangwani L. 2012. The zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy. Hum Mol Genet 21(12):2745-58. PubMed: 22422766 MGI: J:184463
Avila AM; Burnett BG; Taye AA; Gabanella F; Knight MA; Hartenstein P; Cizman Z; Di Prospero NA; Pellizzoni L; Fischbeck KH; Sumner CJ. 2007. Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy. J Clin Invest 117(3):659-71. PubMed: 17318264 MGI: J:120738
Baumer D; Lee S; Nicholson G; Davies JL; Parkinson NJ; Murray LM; Gillingwater TH; Ansorge O; Davies KE; Talbot K. 2009. Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy. PLoS Genet 5(12):e1000773. PubMed: 20019802 MGI: J:161744
Bebee TW; Dominguez CE; Samadzadeh-Tarighat S; Akehurst KL; Chandler DS. 2012. Hypoxia is a modifier of SMN2 splicing and disease severity in a severe SMA mouse model. Hum Mol Genet 21(19):4301-13. PubMed: 22763238 MGI: J:187404
Bevan AK; Hutchinson KR; Foust KD; Braun L; McGovern VL; Schmelzer L; Ward JG; Petruska JC; Lucchesi PA; Burghes AH; Kaspar BK. 2010. Early heart failure in the SMNDelta7 model of spinal muscular atrophy and correction by postnatal scAAV9-SMN delivery. Hum Mol Genet 19(20):3895-905. PubMed: 20639395 MGI: J:164456
Bogdanik LP; Osborne MA; Davis C; Martin WP; Austin A; Rigo F; Bennett CF; Lutz CM. 2015. Systemic, postsymptomatic antisense oligonucleotide rescues motor unit maturation delay in a new mouse model for type II/III spinal muscular atrophy. Proc Natl Acad Sci U S A 112(43):E5863-72. PubMed: 26460027 MGI: J:227184
Bosch-Marce M; Wee CD; Martinez TL; Lipkes CE; Choe DW; Kong L; Van Meerbeke JP; Musaro A; Sumner CJ. 2011. Increased IGF-1 in muscle modulates the phenotype of severe SMA mice. Hum Mol Genet 20(9):1844-53. PubMed: 21325354 MGI: J:170476
Bowerman M; Beauvais A; Anderson CL; Kothary R. 2010. Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model. Hum Mol Genet 19(8):1468-78. PubMed: 20097679 MGI: J:158345
Boyer JG; Deguise MO; Murray LM; Yazdani A; De Repentigny Y; Boudreau-Lariviere C; Kothary R. 2014. Myogenic program dysregulation is contributory to disease pathogenesis in spinal muscular atrophy. Hum Mol Genet 23(16):4249-59. PubMed: 24691550 MGI: J:213423
Boyer JG; Murray LM; Scott K; De Repentigny Y; Renaud JM; Kothary R. 2013. Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy. Skelet Muscle 3(1):24. PubMed: 24119341 MGI: J:202752
Bricceno KV; Martinez T; Leikina E; Duguez S; Partridge TA; Chernomordik LV; Fischbeck KH; Sumner CJ; Burnett BG. 2014. Survival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics. Hum Mol Genet 23(18):4745-57. PubMed: 24760765 MGI: J:213599
Butchbach ME; Edwards JD; Burghes AH. 2007. Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy. Neurobiol Dis 27(2):207-19. PubMed: 17561409 MGI: J:134824
Butchbach ME; Rose FF Jr; Rhoades S; Marston J; McCrone JT; Sinnott R; Lorson CL. 2010. Effect of diet on the survival and phenotype of a mouse model for spinal muscular atrophy. Biochem Biophys Res Commun 391(1):835-40. PubMed: 19945425 MGI: J:156779
Cobb MS; Rose FF; Rindt H; Glascock JJ; Shababi M; Miller MR; Osman EY; Yen PF; Garcia ML; Martin BR; Wetz MJ; Mazzasette C; Feng Z; Ko CP; Lorson CL. 2013. Development and characterization of an SMN2-based intermediate mouse model of Spinal Muscular Atrophy. Hum Mol Genet 22(9):1843-55. PubMed: 23390132 MGI: J:194969
Dachs E; Piedrafita L; Hereu M; Esquerda JE; Caldero J. 2013. Chronic treatment with lithium does not improve neuromuscular phenotype in a mouse model of severe spinal muscular atrophy. Neuroscience 250:417-33. PubMed: 23876328 MGI: J:207041
Dal Mas A; Rogalska ME; Bussani E; Pagani F. 2015. Improvement of SMN2 pre-mRNA processing mediated by exon-specific U1 small nuclear RNA. Am J Hum Genet 96(1):93-103. PubMed: 25557785 MGI: J:230271
Dale JM; Shen H; Barry DM; Garcia VB; Rose FF Jr; Lorson CL; Garcia ML. 2011. The spinal muscular atrophy mouse model, SMADelta7, displays altered axonal transport without global neurofilament alterations. Acta Neuropathol 122(3):331-41. PubMed: 21681521 MGI: J:176036
Dominguez E; Marais T; Chatauret N; Benkhelifa-Ziyyat S; Duque S; Ravassard P; Carcenac R; Astord S; de Moura AP; Voit T; Barkats M. 2011. Intravenous scAAV9 delivery of a codon-optimized SMN1 sequence rescues SMA mice. Hum Mol Genet 20(4):681-93. PubMed: 21118896 MGI: J:168716
Eshraghi M; McFall E; Gibeault S; Kothary R. 2016. Effect of genetic background on the phenotype of the Smn2B/- mouse model of spinal muscular atrophy. Hum Mol Genet :. PubMed: 27538421 MGI: J:238649
Fallini C; Donlin-Asp PG; Rouanet JP; Bassell GJ; Rossoll W. 2016. Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons. J Neurosci 36(13):3811-20. PubMed: 27030765 MGI: J:231749
Farooq F; Abadia-Molina F; Mackenzie D; Hadwen J; Shamim F; O'Reilly S; Holcik M; Mackenzie A. 2013. Celecoxib increases SMN and survival in a severe spinal muscular atrophy mouse model via p38 pathway activation. Hum Mol Genet 22(17):3415-24. PubMed: 23656793 MGI: J:199128
Farooq F; Molina FA; Hadwen J; MacKenzie D; Witherspoon L; Osmond M; Holcik M; MacKenzie A. 2011. Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway. J Clin Invest 121(8):3042-50. PubMed: 21785216 MGI: J:176009
Feng Z; Ling KK; Zhao X; Zhou C; Karp G; Welch EM; Naryshkin N; Ratni H; Chen KS; Metzger F; Paushkin S; Weetall M; Ko CP. 2016. Pharmacologically induced mouse model of adult spinal muscular atrophy to evaluate effectiveness of therapeutics after disease onset. Hum Mol Genet 25(5):964-75. PubMed: 26758873 MGI: J:229461
Fulceri F; Bartalucci A; Paparelli S; Pasquali L; Biagioni F; Ferrucci M; Ruffoli R; Fornai F. 2012. Motor neuron pathology and behavioral alterations at late stages in a SMA mouse model. Brain Res 1442:66-75. PubMed: 22306031 MGI: J:181868
Gavrilina TO; McGovern VL; Workman E; Crawford TO; Gogliotti RG; Didonato CJ; Monani UR; Morris GE; Burghes HM. 2008. Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle specific SMN expression has no phenotypic effect. Hum Mol Genet :. PubMed: 18178576 MGI: J:131663
Gogliotti RG; Lutz C; Jorgensen M; Huebsch K; Koh S; Didonato CJ. 2011. Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice. Neurobiol Dis 43(1):142-51. PubMed: 21396450 MGI: J:174332
Gogliotti RG; Quinlan KA; Barlow CB; Heier CR; Heckman CJ; Didonato CJ. 2012. Motor neuron rescue in spinal muscular atrophy mice demonstrates that sensory-motor defects are a consequence, not a cause, of motor neuron dysfunction. J Neurosci 32(11):3818-29. PubMed: 22423102 MGI: J:183080
Gombash SE; Cowley CJ; Fitzgerald JA; Iyer CC; Fried D; McGovern VL; Williams KC; Burghes AH; Christofi FL; Gulbransen BD; Foust KD. 2015. SMN deficiency disrupts gastrointestinal and enteric nervous system function in mice. Hum Mol Genet 24(13):3847-60. PubMed: 25859009 MGI: J:223348
Hayhurst M; Wagner AK; Cerletti M; Wagers AJ; Rubin LL. 2012. A cell-autonomous defect in skeletal muscle satellite cells expressing low levels of survival of motor neuron protein. Dev Biol 368(2):323-34. PubMed: 22705478 MGI: J:186551
Heier CR; Satta R; Lutz C; DiDonato CJ. 2010. Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice. Hum Mol Genet 19(20):3906-18. PubMed: 20693262 MGI: J:164446
Hunter G; Aghamaleky Sarvestany A; Roche SL; Symes RC; Gillingwater TH. 2014. SMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy. Hum Mol Genet 23(9):2235-50. PubMed: 24301677 MGI: J:208449
Huo Q; Kayikci M; Odermatt P; Meyer K; Michels O; Saxena S; Ule J; Schumperli D. 2014. Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: evidence for involvement of splicing regulatory proteins. RNA Biol 11(11):1430-46. PubMed: 25692239 MGI: J:231140
Iyer CC; McGovern VL; Murray JD; Gombash SE; Zaworski PG; Foust KD; Janssen PM; Burghes AH. 2015. Low levels of Survival Motor Neuron protein are sufficient for normal muscle function in the SMNDelta7 mouse model of SMA. Hum Mol Genet 24(21):6160-73. PubMed: 26276812 MGI: J:226174
Jablonka S; Beck M; Lechner BD; Mayer C; Sendtner M. 2007. Defective Ca2+ channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophy. J Cell Biol 179(1):139-49. PubMed: 17923533 MGI: J:134807
Jablonka S; Karle K; Sandner B; Andreassi C; von Au K; Sendtner M. 2006. Distinct and overlapping alterations in motor and sensory neurons in a mouse model of spinal muscular atrophy. Hum Mol Genet 15(3):511-8. PubMed: 16396995 MGI: J:105422
Kariya S; Obis T; Garone C; Akay T; Sera F; Iwata S; Homma S; Monani UR. 2014. Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation. J Clin Invest 124(2):785-800. PubMed: 24463453 MGI: J:208442
Kariya S; Park GH; Maeno-Hikichi Y; Leykekhman O; Lutz C; Arkovitz MS; Landmesser LT; Monani UR. 2008. Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy. Hum Mol Genet 17(16):2552-69. PubMed: 18492800 MGI: J:138437
Khairallah MT; Astroski J; Custer SK; Androphy EJ; Franklin CL; Lorson CL. 2017. SMN deficiency negatively impacts red pulp macrophages and spleen development in mouse models of spinal muscular atrophy. Hum Mol Genet 26(5):932-941. PubMed: 28062667 MGI: J:241426
Kong L; Wang X; Choe DW; Polley M; Burnett BG; Bosch-Marce M; Griffin JW; Rich MM; Sumner CJ. 2009. Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice. J Neurosci 29(3):842-51. PubMed: 19158308 MGI: J:144843
Kye MJ; Niederst ED; Wertz MH; Goncalves Ido C; Akten B; Dover KZ; Peters M; Riessland M; Neveu P; Wirth B; Kosik KS; Sardi SP; Monani UR; Passini MA; Sahin M. 2014. SMN regulates axonal local translation via miR-183/mTOR pathway. Hum Mol Genet 23(23):6318-31. PubMed: 25055867 MGI: J:215597
Le TT; Pham LT; Butchbach ME; Zhang HL; Monani UR; Coovert DD; Gavrilina TO; Xing L; Bassell GJ; Burghes AH. 2005. SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN. Hum Mol Genet 14(6):845-57. PubMed: 15703193 MGI: J:97103
Lee AJ; Awano T; Park GH; Monani UR. 2012. Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy. PLoS One 7(9):e46353. PubMed: 23029491 MGI: J:191961
Lee YI; Mikesh M; Smith I; Rimer M; Thompson W. 2011. Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons. Dev Biol 356(2):432-44. PubMed: 21658376 MGI: J:175468
Ling KK; Gibbs RM; Feng Z; Ko CP. 2012. Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy. Hum Mol Genet 21(1):185-95. PubMed: 21968514 MGI: J:178856
Ling KK; Lin MY; Zingg B; Feng Z; Ko CP. 2010. Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophy. PLoS One 5(11):e15457. PubMed: 21085654 MGI: J:166818
Lotti F; Imlach WL; Saieva L; Beck ES; Hao le T; Li DK; Jiao W; Mentis GZ; Beattie CE; McCabe BD; Pellizzoni L. 2012. An SMN-Dependent U12 Splicing Event Essential for Motor Circuit Function. Cell 151(2):440-54. PubMed: 23063131 MGI: J:189067
Luchetti A; Ciafre SA; Murdocca M; Malgieri A; Masotti A; Sanchez M; Farace MG; Novelli G; Sangiuolo F. 2015. A Perturbed MicroRNA Expression Pattern Characterizes Embryonic Neural Stem Cells Derived from a Severe Mouse Model of Spinal Muscular Atrophy (SMA). Int J Mol Sci 16(8):18312-27. PubMed: 26258776 MGI: J:238913
Lutz CM; Kariya S; Patruni S; Osborne MA; Liu D; Henderson CE; Li DK; Pellizzoni L; Rojas J; Valenzuela DM; Murphy AJ; Winberg ML; Monani UR. 2011. Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy. J Clin Invest 121(8):3029-41. PubMed: 21785219 MGI: J:176007
McGivern JV; Patitucci TN; Nord JA; Barabas ME; Stucky CL; Ebert AD. 2013. Spinal muscular atrophy astrocytes exhibit abnormal calcium regulation and reduced growth factor production. Glia 61(9):1418-28. PubMed: 23839956 MGI: J:199444
McGovern VL; Gavrilina TO; Beattie CE; Burghes AH. 2008. Embryonic motor axon development in the severe SMA mouse. Hum Mol Genet 17(18):2900-9. PubMed: 18603534 MGI: J:138317
McGovern VL; Massoni-Laporte A; Wang X; Le TT; Le HT; Beattie CE; Rich MM; Burghes AH. 2015. Plastin 3 Expression Does Not Modify Spinal Muscular Atrophy Severity in the 7 SMA Mouse. PLoS One 10(7):e0132364. PubMed: 26134627 MGI: J:238311
Meyer K; Marquis J; Trub J; Nlend Nlend R; Verp S; Ruepp MD; Imboden H; Barde I; Trono D; Schumperli D. 2009. Rescue of a severe mouse model for spinal muscular atrophy by U7 snRNA-mediated splicing modulation. Hum Mol Genet 18(3):546-55. PubMed: 19010792 MGI: J:143540
Miller N; Feng Z; Edens BM; Yang B; Shi H; Sze CC; Hong BT; Su SC; Cantu JA; Topczewski J; Crawford TO; Ko CP; Sumner CJ; Ma L; Ma YC. 2015. Non-aggregating tau phosphorylation by cyclin-dependent kinase 5 contributes to motor neuron degeneration in spinal muscular atrophy. J Neurosci 35(15):6038-50. PubMed: 25878277 MGI: J:221676
Miller N; Shi H; Zelikovich AS; Ma YC. 2016. Motor neuron mitochondrial dysfunction in spinal muscular atrophy. Hum Mol Genet 25(16):3395-3406. PubMed: 27488123 MGI: J:237935
Monani UR; Pastore MT; Gavrilina TO; Jablonka S; Le TT; Andreassi C; DiCocco JM; Lorson C; Androphy EJ; Sendtner M; Podell M; Burghes AH. 2003. A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy. J Cell Biol 160(1):41-52. PubMed: 12515823 MGI: J:81238
Monani UR; Sendtner M; Coovert DD; Parsons DW; Andreassi C; Le TT; Jablonka S; Schrank B; Rossol W; Prior TW; Morris GE; Burghes AH. 2000. The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophy. Hum Mol Genet 9(3):333-9. PubMed: 10655541 MGI: J:60592
Murray LM; Comley LH; Thomson D; Parkinson N; Talbot K; Gillingwater TH. 2008. Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy. Hum Mol Genet 17(7):949-62. PubMed: 18065780 MGI: J:132467
Murray LM; Lee S; Baumer D; Parson SH; Talbot K; Gillingwater TH. 2009. Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy. Hum Mol Genet :. PubMed: 19884170 MGI: J:155336
Mutsaers CA; Wishart TM; Lamont DJ; Riessland M; Schreml J; Comley LH; Murray LM; Parson SH; Lochmuller H; Wirth B; Talbot K; Gillingwater TH. 2011. Reversible molecular pathology of skeletal muscle in spinal muscular atrophy. Hum Mol Genet 20(22):4334-44. PubMed: 21840928 MGI: J:176892
Neve A; Trub J; Saxena S; Schumperli D. 2016. Central and peripheral defects in motor units of the diaphragm of spinal muscular atrophy mice. Mol Cell Neurosci 70:30-41. PubMed: 26621405 MGI: J:231139
Nolle A; Zeug A; van Bergeijk J; Tonges L; Gerhard R; Brinkmann H; Al Rayes S; Hensel N; Schill Y; Apkhazava D; Jablonka S; O Fmer J; Kumar Srivastav R; Baasner A; Lingor P; Wirth B; Ponimaskin E; Niedenthal R; Grothe C; Claus P. 2011. The spinal muscular atrophy disease protein SMN is linked to the rho-kinase pathway via profilin. Hum Mol Genet :. PubMed: 21920940 MGI: J:177764
Novoyatleva T; Heinrich B; Tang Y; Benderska N; Butchbach ME; Lorson CL; Lorson MA; Ben-Dov C; Fehlbaum P; Bracco L; Burghes AH; Bollen M; Stamm S. 2008. Protein phosphatase 1 binds to the RNA recognition motif of several splicing factors and regulates alternative pre-mRNA processing. Hum Mol Genet 17(1):52-70. PubMed: 17913700 MGI: J:130114
Osman EY; Miller MR; Robbins KL; Lombardi AM; Atkinson AK; Brehm AJ; Lorson CL. 2014. Morpholino antisense oligonucleotides targeting intronic repressor Element1 improve phenotype in SMA mouse models. Hum Mol Genet 23(18):4832-45. PubMed: 24781211 MGI: J:213594
Paez-Colasante X; Seaberg B; Martinez TL; Kong L; Sumner CJ; Rimer M. 2013. Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons. PLoS One 8(9):e75866. PubMed: 24086650 MGI: J:206015
Pagliarini V; Pelosi L; Bustamante MB; Nobili A; Berardinelli MG; D'Amelio M; Musaro A; Sette C. 2015. SAM68 is a physiological regulator of SMN2 splicing in spinal muscular atrophy. J Cell Biol 211(1):77-90. PubMed: 26438828 MGI: J:227647
Park GH; Maeno-Hikichi Y; Awano T; Landmesser LT; Monani UR. 2010. Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene. J Neurosci 30(36):12005-19. PubMed: 20826664 MGI: J:164292
Porensky PN; Mitrpant C; McGovern VL; Bevan AK; Foust KD; Kaspar BK; Wilton SD; Burghes AH. 2012. A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse. Hum Mol Genet 21(7):1625-38. PubMed: 22186025 MGI: J:181560
Riessland M; Ackermann B; Forster A; Jakubik M; Hauke J; Garbes L; Fritzsche I; Mende Y; Blumcke I; Hahnen E; Wirth B. 2010. SAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophy. Hum Mol Genet 19(8):1492-506. PubMed: 20097677 MGI: J:158347
Robbins KL; Glascock JJ; Osman EY; Miller MR; Lorson CL. 2014. Defining the therapeutic window in a severe animal model of spinal muscular atrophy. Hum Mol Genet 23(17):4559-68. PubMed: 24722206 MGI: J:213616
Rose FF Jr; Mattis VB; Rindt H; Lorson CL. 2009. Delivery of recombinant follistatin lessens disease severity in a mouse model of spinal muscular atrophy. Hum Mol Genet 18(6):997-1005. PubMed: 19074460 MGI: J:145746
Rossoll W; Jablonka S; Andreassi C; Kroning AK; Karle K; Monani UR; Sendtner M. 2003. Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneurons. J Cell Biol 163(4):801-12. PubMed: 14623865 MGI: J:86712
Ruggiu M; McGovern VL; Lotti F; Saieva L; Li DK; Kariya S; Monani UR; Burghes AH; Pellizzoni L. 2012. A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy. Mol Cell Biol 32(1):126-38. PubMed: 22037760 MGI: J:183557
Ruiz R; Casanas JJ; Torres-Benito L; Cano R; Tabares L. 2010. Altered intracellular Ca2+ homeostasis in nerve terminals of severe spinal muscular atrophy mice. J Neurosci 30(3):849-57. PubMed: 20089893 MGI: J:157700
Sanchez G; Dury AY; Murray LM; Biondi O; Tadesse H; El Fatimy R; Kothary R; Charbonnier F; Khandjian EW; Cote J. 2013. A novel function for the survival motoneuron protein as a translational regulator. Hum Mol Genet 22(4):668-84. PubMed: 23136128 MGI: J:191211
See K; Yadav P; Giegerich M; Cheong PS; Graf M; Vyas H; Lee SG; Mathavan S; Fischer U; Sendtner M; Winkler C. 2014. SMN deficiency alters Nrxn2 expression and splicing in zebrafish and mouse models of spinal muscular atrophy. Hum Mol Genet 23(7):1754-70. PubMed: 24218366 MGI: J:207140
Shababi M; Habibi J; Yang HT; Vale SM; Sewell WA; Lorson CL. 2010. Cardiac defects contribute to the pathology of spinal muscular atrophy models. Hum Mol Genet 19(20):4059-71. PubMed: 20696672 MGI: J:164444
Sleigh JN; Gillingwater TH; Talbot K. 2011. The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy. Dis Model Mech 4(4):457-67. PubMed: 21708901 MGI: J:175452
Subramanian N; Wetzel A; Dombert B; Yadav P; Havlicek S; Jablonka S; Nassar MA; Blum R; Sendtner M. 2012. Role of Nav1.9 in activity-dependent axon growth in motoneurons. Hum Mol Genet 21(16):3655-67. PubMed: 22641814 MGI: J:185985
Sumner CJ; Wee CD; Warsing LC; Choe DW; Ng AS; Lutz C; Wagner KR. 2009. Inhibition of myostatin does not ameliorate disease features of severe spinal muscular atrophy mice. Hum Mol Genet 18(17):3145-52. PubMed: 19477958 MGI: J:151438
Taylor AS; Glascock JJ; Rose FF Jr; Lutz C; Lorson CL. 2013. Restoration of SMN to Emx-1 expressing cortical neurons is not sufficient to provide benefit to a severe mouse model of Spinal Muscular Atrophy. Transgenic Res 22(5):1029-36. PubMed: 23512182 MGI: J:213313
Tejero R; Lopez-Manzaneda M; Arumugam S; Tabares L. 2016. Synaptotagmin-2, and -1, linked to neurotransmission impairment and vulnerability in Spinal Muscular Atrophy. Hum Mol Genet :. PubMed: 27577871 MGI: J:238645
Thomson SR; Nahon JE; Mutsaers CA; Thomson D; Hamilton G; Parson SH; Gillingwater TH. 2012. Morphological characteristics of motor neurons do not determine their relative susceptibility to degeneration in a mouse model of severe spinal muscular atrophy. PLoS One 7(12):e52605. PubMed: 23285108 MGI: J:195758
Tisdale S; Lotti F; Saieva L; Van Meerbeke JP; Crawford TO; Sumner CJ; Mentis GZ; Pellizzoni L. 2013. SMN Is Essential for the Biogenesis of U7 Small Nuclear Ribonucleoprotein and 3'-End Formation of Histone mRNAs. Cell Rep 5(5):1187-95. PubMed: 24332368 MGI: J:204134
Torres-Benito L; Neher MF; Cano R; Ruiz R; Tabares L. 2011. SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals. PLoS One 6(10):e26164. PubMed: 22022549 MGI: J:179582
Turner BJ; Parkinson NJ; Davies KE; Talbot K. 2009. Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model. Neurobiol Dis 34(3):511-7. PubMed: 19332122 MGI: J:150474
Valsecchi V; Boido M; De Amicis E; Piras A; Vercelli A. 2015. Expression of Muscle-Specific MiRNA 206 in the Progression of Disease in a Murine SMA Model. PLoS One 10(6):e0128560. PubMed: 26030275 MGI: J:237725
Van Meerbeke JP; Gibbs RM; Plasterer HL; Miao W; Feng Z; Lin MY; Rucki AA; Wee CD; Xia B; Sharma S; Jacques V; Li DK; Pellizzoni L; Rusche JR; Ko CP; Sumner CJ. 2013. The DcpS inhibitor RG3039 improves motor function in SMA mice. Hum Mol Genet 22(20):4074-83. PubMed: 23727836 MGI: J:201100
Voigt T; Meyer K; Baum O; Schumperli D. 2010. Ultrastructural changes in diaphragm neuromuscular junctions in a severe mouse model for Spinal Muscular Atrophy and their prevention by bifunctional U7 snRNA correcting SMN2 splicing. Neuromuscul Disord 20(11):744-52. PubMed: 20832308 MGI: J:231141
Walker MP; Rajendra TK; Saieva L; Fuentes JL; Pellizzoni L; Matera AG. 2008. SMN complex localizes to the sarcomeric Z-disc and is a proteolytic target of calpain. Hum Mol Genet 17(21):3399-410. PubMed: 18689355 MGI: J:140332
Wishart TM; Huang JP; Murray LM; Lamont DJ; Mutsaers CA; Ross J; Geldsetzer P; Ansorge O; Talbot K; Parson SH; Gillingwater TH. 2010. SMN deficiency disrupts brain development in a mouse model of severe spinal muscular atrophy. Hum Mol Genet 19(21):4216-28. PubMed: 20705736 MGI: J:164890
Wishart TM; Mutsaers CA; Riessland M; Reimer MM; Hunter G; Hannam ML; Eaton SL; Fuller HR; Roche SL; Somers E; Morse R; Young PJ; Lamont DJ; Hammerschmidt M; Joshi A; Hohenstein P; Morris GE; Parson SH; Skehel PA; Becker T; Robinson IM; Becker CG; Wirth B; Gillingwater TH. 2014. Dysregulation of ubiquitin homeostasis and beta-catenin signaling promote spinal muscular atrophy. J Clin Invest 124(4):1821-34. PubMed: 24590288 MGI: J:209625
Workman E; Saieva L; Carrel TL; Crawford TO; Liu D; Lutz C; Beattie CE; Pellizzoni L; Burghes AH. 2009. A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice. Hum Mol Genet 18(12):2215-29. PubMed: 19329542 MGI: J:148541
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d'Errico P; Boido M; Piras A; Valsecchi V; De Amicis E; Locatelli D; Capra S; Vagni F; Vercelli A; Battaglia G. 2013. Selective vulnerability of spinal and cortical motor neuron subpopulations in delta7 SMA mice. PLoS One 8(12):e82654. PubMed: 24324819 MGI: J:209739

Additional - Smn1^tm1Msd related

Ahmad S; Wang Y; Shaik GM; Burghes AH; Gangwani L. 2012. The zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy. Hum Mol Genet 21(12):2745-58. PubMed: 22422766 MGI: J:184463
Balasubramaniam V; Ryan SL; Seedorf GJ; Roth EV; Heumann TR; Yoder MC; Ingram DA; Hogan CJ; Markham NE; Abman SH. 2010. Bone marrow-derived angiogenic cells restore lung alveolar and vascular structure after neonatal hyperoxia in infant mice. Am J Physiol Lung Cell Mol Physiol 298(3):L315-23. PubMed: 20008116 MGI: J:157669
Baumer D; Lee S; Nicholson G; Davies JL; Parkinson NJ; Murray LM; Gillingwater TH; Ansorge O; Davies KE; Talbot K. 2009. Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy. PLoS Genet 5(12):e1000773. PubMed: 20019802 MGI: J:161744
Bebee TW; Dominguez CE; Samadzadeh-Tarighat S; Akehurst KL; Chandler DS. 2012. Hypoxia is a modifier of SMN2 splicing and disease severity in a severe SMA mouse model. Hum Mol Genet 21(19):4301-13. PubMed: 22763238 MGI: J:187404
Bevan AK; Hutchinson KR; Foust KD; Braun L; McGovern VL; Schmelzer L; Ward JG; Petruska JC; Lucchesi PA; Burghes AH; Kaspar BK. 2010. Early heart failure in the SMNDelta7 model of spinal muscular atrophy and correction by postnatal scAAV9-SMN delivery. Hum Mol Genet 19(20):3895-905. PubMed: 20639395 MGI: J:164456
Bogdanik LP; Osborne MA; Davis C; Martin WP; Austin A; Rigo F; Bennett CF; Lutz CM. 2015. Systemic, postsymptomatic antisense oligonucleotide rescues motor unit maturation delay in a new mouse model for type II/III spinal muscular atrophy. Proc Natl Acad Sci U S A 112(43):E5863-72. PubMed: 26460027 MGI: J:227184
Bordet T. 2009. Generation of an SMN2 transgene (line 11) MGI Direct Data Submission :. MGI: J:144852
Bordet T. 2009. Generation of an SMN2 transgene (line 46) MGI Direct Data Submission :. MGI: J:144853
Bosch-Marce M; Wee CD; Martinez TL; Lipkes CE; Choe DW; Kong L; Van Meerbeke JP; Musaro A; Sumner CJ. 2011. Increased IGF-1 in muscle modulates the phenotype of severe SMA mice. Hum Mol Genet 20(9):1844-53. PubMed: 21325354 MGI: J:170476
Bowerman M; Anderson CL; Beauvais A; Boyl PP; Witke W; Kothary R. 2009. SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesis. Mol Cell Neurosci 42(1):66-74. PubMed: 19497369 MGI: J:154248
Bowerman M; Beauvais A; Anderson CL; Kothary R. 2010. Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model. Hum Mol Genet 19(8):1468-78. PubMed: 20097679 MGI: J:158345
Bowerman M; Michalski JP; Beauvais A; Murray LM; DeRepentigny Y; Kothary R. 2014. Defects in pancreatic development and glucose metabolism in SMN-depleted mice independent of canonical spinal muscular atrophy neuromuscular pathology. Hum Mol Genet 23(13):3432-44. PubMed: 24497575 MGI: J:210674
Boyer JG; Deguise MO; Murray LM; Yazdani A; De Repentigny Y; Boudreau-Lariviere C; Kothary R. 2014. Myogenic program dysregulation is contributory to disease pathogenesis in spinal muscular atrophy. Hum Mol Genet 23(16):4249-59. PubMed: 24691550 MGI: J:213423
Boyer JG; Murray LM; Scott K; De Repentigny Y; Renaud JM; Kothary R. 2013. Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy. Skelet Muscle 3(1):24. PubMed: 24119341 MGI: J:202752
Bricceno KV; Martinez T; Leikina E; Duguez S; Partridge TA; Chernomordik LV; Fischbeck KH; Sumner CJ; Burnett BG. 2014. Survival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics. Hum Mol Genet 23(18):4745-57. PubMed: 24760765 MGI: J:213599
Butchbach ME; Edwards JD; Burghes AH. 2007. Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy. Neurobiol Dis 27(2):207-19. PubMed: 17561409 MGI: J:134824
Butchbach ME; Rose FF Jr; Rhoades S; Marston J; McCrone JT; Sinnott R; Lorson CL. 2010. Effect of diet on the survival and phenotype of a mouse model for spinal muscular atrophy. Biochem Biophys Res Commun 391(1):835-40. PubMed: 19945425 MGI: J:156779
Cobb MS; Rose FF; Rindt H; Glascock JJ; Shababi M; Miller MR; Osman EY; Yen PF; Garcia ML; Martin BR; Wetz MJ; Mazzasette C; Feng Z; Ko CP; Lorson CL. 2013. Development and characterization of an SMN2-based intermediate mouse model of Spinal Muscular Atrophy. Hum Mol Genet 22(9):1843-55. PubMed: 23390132 MGI: J:194969
Comley LH; Nijssen J; Frost-Nylen J; Hedlund E. 2016. Cross-disease comparison of amyotrophic lateral sclerosis and spinal muscular atrophy reveals conservation of selective vulnerability but differential neuromuscular junction pathology. J Comp Neurol 524(7):1424-42. PubMed: 26502195 MGI: J:231487
Dachs E; Piedrafita L; Hereu M; Esquerda JE; Caldero J. 2013. Chronic treatment with lithium does not improve neuromuscular phenotype in a mouse model of severe spinal muscular atrophy. Neuroscience 250:417-33. PubMed: 23876328 MGI: J:207041
Dal Mas A; Rogalska ME; Bussani E; Pagani F. 2015. Improvement of SMN2 pre-mRNA processing mediated by exon-specific U1 small nuclear RNA. Am J Hum Genet 96(1):93-103. PubMed: 25557785 MGI: J:230271
Dale JM; Shen H; Barry DM; Garcia VB; Rose FF Jr; Lorson CL; Garcia ML. 2011. The spinal muscular atrophy mouse model, SMADelta7, displays altered axonal transport without global neurofilament alterations. Acta Neuropathol 122(3):331-41. PubMed: 21681521 MGI: J:176036
Deguise MO; De Repentigny Y; McFall E; Auclair N; Sad S; Kothary R. 2017. Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy mice. Hum Mol Genet 26(4):801-819. PubMed: 28108555 MGI: J:241662
Dombert B; Sivadasan R; Simon CM; Jablonka S; Sendtner M. 2014. Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons. PLoS One 9(10):e110846. PubMed: 25338097 MGI: J:222448
Dominguez E; Marais T; Chatauret N; Benkhelifa-Ziyyat S; Duque S; Ravassard P; Carcenac R; Astord S; de Moura AP; Voit T; Barkats M. 2011. Intravenous scAAV9 delivery of a codon-optimized SMN1 sequence rescues SMA mice. Hum Mol Genet 20(4):681-93. PubMed: 21118896 MGI: J:168716
El-Khodor BF; Edgar N; Chen A; Winberg ML; Joyce C; Brunner D; Suarez-Farinas M; Heyes MP. 2008. Identification of a battery of tests for drug candidate evaluation in the SMNDelta7 neonate model of spinal muscular atrophy. Exp Neurol 212(1):29-43. PubMed: 18455159 MGI: J:137949
Eshraghi M; McFall E; Gibeault S; Kothary R. 2016. Effect of genetic background on the phenotype of the Smn2B/- mouse model of spinal muscular atrophy. Hum Mol Genet :. PubMed: 27538421 MGI: J:238649
Fallini C; Donlin-Asp PG; Rouanet JP; Bassell GJ; Rossoll W. 2016. Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons. J Neurosci 36(13):3811-20. PubMed: 27030765 MGI: J:231749
Fallini C; Rouanet JP; Donlin-Asp PG; Guo P; Zhang H; Singer RH; Rossoll W; Bassell GJ. 2014. Dynamics of survival of motor neuron (SMN) protein interaction with the mRNA-binding protein IMP1 facilitates its trafficking into motor neuron axons. Dev Neurobiol 74(3):319-32. PubMed: 23897586 MGI: J:238830
Farooq F; Abadia-Molina F; Mackenzie D; Hadwen J; Shamim F; O'Reilly S; Holcik M; Mackenzie A. 2013. Celecoxib increases SMN and survival in a severe spinal muscular atrophy mouse model via p38 pathway activation. Hum Mol Genet 22(17):3415-24. PubMed: 23656793 MGI: J:199128
Farooq F; Molina FA; Hadwen J; MacKenzie D; Witherspoon L; Osmond M; Holcik M; MacKenzie A. 2011. Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway. J Clin Invest 121(8):3042-50. PubMed: 21785216 MGI: J:176009
Feng Z; Ling KK; Zhao X; Zhou C; Karp G; Welch EM; Naryshkin N; Ratni H; Chen KS; Metzger F; Paushkin S; Weetall M; Ko CP. 2016. Pharmacologically induced mouse model of adult spinal muscular atrophy to evaluate effectiveness of therapeutics after disease onset. Hum Mol Genet 25(5):964-75. PubMed: 26758873 MGI: J:229461
Fulceri F; Bartalucci A; Paparelli S; Pasquali L; Biagioni F; Ferrucci M; Ruffoli R; Fornai F. 2012. Motor neuron pathology and behavioral alterations at late stages in a SMA mouse model. Brain Res 1442:66-75. PubMed: 22306031 MGI: J:181868
Gavrilina TO; McGovern VL; Workman E; Crawford TO; Gogliotti RG; Didonato CJ; Monani UR; Morris GE; Burghes HM. 2008. Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle specific SMN expression has no phenotypic effect. Hum Mol Genet :. PubMed: 18178576 MGI: J:131663
Genabai NK; Ahmad S; Zhang Z; Jiang X; Gabaldon CA; Gangwani L. 2015. Genetic inhibition of JNK3 ameliorates spinal muscular atrophy. Hum Mol Genet 24(24):6986-7004. PubMed: 26423457 MGI: J:226840
Gladman JT; Bebee TW; Edwards C; Wang X; Sahenk Z; Rich MM; Chandler DS. 2010. A humanized Smn gene containing the SMN2 nucleotide alteration in exon 7 mimics SMN2 splicing and the SMA disease phenotype. Hum Mol Genet 19(21):4239-52. PubMed: 20705738 MGI: J:164889
Gogliotti RG; Cardona H; Singh J; Bail S; Emery C; Kuntz N; Jorgensen M; Durens M; Xia B; Barlow C; Heier CR; Plasterer HL; Jacques V; Kiledjian M; Jarecki J; Rusche J; Didonato CJ. 2013. The DcpS inhibitor RG3039 improves survival, function and motor unit pathologies in two SMA mouse models. Hum Mol Genet 22(20):4084-101. PubMed: 23736298 MGI: J:201094
Gogliotti RG; Lutz C; Jorgensen M; Huebsch K; Koh S; Didonato CJ. 2011. Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice. Neurobiol Dis 43(1):142-51. PubMed: 21396450 MGI: J:174332
Gogliotti RG; Quinlan KA; Barlow CB; Heier CR; Heckman CJ; Didonato CJ. 2012. Motor neuron rescue in spinal muscular atrophy mice demonstrates that sensory-motor defects are a consequence, not a cause, of motor neuron dysfunction. J Neurosci 32(11):3818-29. PubMed: 22423102 MGI: J:183080
Gombash SE; Cowley CJ; Fitzgerald JA; Iyer CC; Fried D; McGovern VL; Williams KC; Burghes AH; Christofi FL; Gulbransen BD; Foust KD. 2015. SMN deficiency disrupts gastrointestinal and enteric nervous system function in mice. Hum Mol Genet 24(13):3847-60. PubMed: 25859009 MGI: J:223348
Hayhurst M; Wagner AK; Cerletti M; Wagers AJ; Rubin LL. 2012. A cell-autonomous defect in skeletal muscle satellite cells expressing low levels of survival of motor neuron protein. Dev Biol 368(2):323-34. PubMed: 22705478 MGI: J:186551
Heier CR; Satta R; Lutz C; DiDonato CJ. 2010. Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice. Hum Mol Genet 19(20):3906-18. PubMed: 20693262 MGI: J:164446
Hunter G; Aghamaleky Sarvestany A; Roche SL; Symes RC; Gillingwater TH. 2014. SMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy. Hum Mol Genet 23(9):2235-50. PubMed: 24301677 MGI: J:208449
Huo Q; Kayikci M; Odermatt P; Meyer K; Michels O; Saxena S; Ule J; Schumperli D. 2014. Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: evidence for involvement of splicing regulatory proteins. RNA Biol 11(11):1430-46. PubMed: 25692239 MGI: J:231140
Iyer CC; McGovern VL; Murray JD; Gombash SE; Zaworski PG; Foust KD; Janssen PM; Burghes AH. 2015. Low levels of Survival Motor Neuron protein are sufficient for normal muscle function in the SMNDelta7 mouse model of SMA. Hum Mol Genet 24(21):6160-73. PubMed: 26276812 MGI: J:226174
Jablonka S; Beck M; Lechner BD; Mayer C; Sendtner M. 2007. Defective Ca2+ channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophy. J Cell Biol 179(1):139-49. PubMed: 17923533 MGI: J:134807
Jablonka S; Holtmann B; Meister G; Bandilla M; Rossoll W; Fischer U; Sendtner M. 2002. Gene targeting of Gemin2 in mice reveals a correlation between defects in the biogenesis of U snRNPs and motoneuron cell death. Proc Natl Acad Sci U S A 99(15):10126-31. PubMed: 12091709 MGI: J:81784
Jablonka S; Karle K; Sandner B; Andreassi C; von Au K; Sendtner M. 2006. Distinct and overlapping alterations in motor and sensory neurons in a mouse model of spinal muscular atrophy. Hum Mol Genet 15(3):511-8. PubMed: 16396995 MGI: J:105422
Jablonka S; Schrank B; Kralewski M; Rossoll W; Sendtner M. 2000. Reduced survival motor neuron (Smn) gene dose in mice leads to motor neuron degeneration: an animal model for spinal muscular atrophy type III. Hum Mol Genet 9(3):341-6. PubMed: 10655542 MGI: J:60591
Kariya S; Obis T; Garone C; Akay T; Sera F; Iwata S; Homma S; Monani UR. 2014. Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation. J Clin Invest 124(2):785-800. PubMed: 24463453 MGI: J:208442
Kariya S; Park GH; Maeno-Hikichi Y; Leykekhman O; Lutz C; Arkovitz MS; Landmesser LT; Monani UR. 2008. Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy. Hum Mol Genet 17(16):2552-69. PubMed: 18492800 MGI: J:138437
Kariya S; Re DB; Jacquier A; Nelson K; Przedborski S; Monani UR. 2012. Mutant superoxide dismutase 1 (SOD1), a cause of amyotrophic lateral sclerosis, disrupts the recruitment of SMN, the spinal muscular atrophy protein to nuclear Cajal bodies. Hum Mol Genet 21(15):3421-34. PubMed: 22581780 MGI: J:185362
Khairallah MT; Astroski J; Custer SK; Androphy EJ; Franklin CL; Lorson CL. 2017. SMN deficiency negatively impacts red pulp macrophages and spleen development in mouse models of spinal muscular atrophy. Hum Mol Genet 26(5):932-941. PubMed: 28062667 MGI: J:241426
Kong L; Wang X; Choe DW; Polley M; Burnett BG; Bosch-Marce M; Griffin JW; Rich MM; Sumner CJ. 2009. Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice. J Neurosci 29(3):842-51. PubMed: 19158308 MGI: J:144843
Kwon DY; Motley WW; Fischbeck KH; Burnett BG. 2011. Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice. Hum Mol Genet 20(18):3667-77. PubMed: 21693563 MGI: J:174791
Kye MJ; Niederst ED; Wertz MH; Goncalves Ido C; Akten B; Dover KZ; Peters M; Riessland M; Neveu P; Wirth B; Kosik KS; Sardi SP; Monani UR; Passini MA; Sahin M. 2014. SMN regulates axonal local translation via miR-183/mTOR pathway. Hum Mol Genet 23(23):6318-31. PubMed: 25055867 MGI: J:215597
Le TT; McGovern VL; Alwine IE; Wang X; Massoni-Laporte A; Rich MM; Burghes AH. 2011. Temporal requirement for high SMN expression in SMA mice. Hum Mol Genet 20(18):3578-91. PubMed: 21672919 MGI: J:174960
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Also Known As: SMN2 SMN- HB9-eGFP

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Grm7Tg(SMN2)89Ahmb

Allele Symbol: Grm7Tg(SMN2)89Ahmb

Smn1tm1Msd

Allele Symbol: Smn1tm1Msd

Tg(Hlxb9-GFP)1Tmj

Allele Symbol: Tg(Hlxb9-GFP)1Tmj

Disease Terms

Research Areas By Genotype

This mouse can be used to support research in many areas including:

Genotype: GFP related

Genotype: Smn1tm1Msd related

References

Additional - Grm7Tg(SMN2)89Ahmb related

Additional - Smn1tm1Msd related

Additional - Tg(Hlxb9-GFP)1Tmj related

Genotyping Protocols

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Citation

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Live Mouse

Cryorecovery - Pricing

Progeny testing is not required

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No Liability

Grm7^{Tg(SMN2)89Ahmb}

Allele Symbol: Grm7^{Tg(SMN2)89Ahmb}

Smn1^tm1Msd

Allele Symbol: Smn1^tm1Msd

Genotype: Smn1^tm1Msd related

Additional - Grm7^{Tg(SMN2)89Ahmb} related

Additional - Smn1^tm1Msd related