JAX
Mouse Strain Datasheet - 006570
STOCK Grm7Tg(SMN2)89Ahmb Smn1tm1Msd Tg(Hlxb9-GFP)1Tmj/J
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).
Read More +Genetic overview
| Genetic Background | Generation |
|---|---|
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Grm7Tg(SMN2)89Ahmb
| Allele Type |
|---|
| Transgenic (Humanized sequence, Hypomorph, Inserted expressed sequence) |
Smn1tm1Msd
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Targeted (Null/Knockout, Reporter) | Smn1 | survival motor neuron 1 |
Tg(Hlxb9-GFP)1Tmj
| Allele Type |
|---|
| Transgenic (Reporter) |
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 Smn1tm1Msd 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
Grm7Tg(SMN2)89Ahmb
Allele Symbol: Grm7Tg(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 |
Smn1tm1Msd
Allele Symbol: Smn1tm1Msd
| 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 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: Smn1tm1Msd related
- Neurobiology Research
- Spinal Muscular Atrophy (SMA)
References
Additional - Grm7Tg(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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . MGI: J:170476
- Bowerman M; Beauvais A; Anderson CL; Kothary R. 2010. Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . MGI: J:213599
- Butchbach ME; Edwards JD; Burghes AH. 2007. Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . MGI: J:241426
- Kim JK; Caine C; Awano T; Herbst R; Monani UR. 2017. Motor neuronal repletion of the NMJ organizer, Agrin, modulates the severity of the spinal muscular atrophy disease phenotype in model mice.. In: . . MGI: J:242834
- 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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).. In: . . 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.. In: . . 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.. In: . . MGI: J:199444
- McGovern VL; Gavrilina TO; Beattie CE; Burghes AH. 2008. Embryonic motor axon development in the severe SMA mouse.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . MGI: J:221676
- Miller N; Shi H; Zelikovich AS; Ma YC. 2016. Motor neuron mitochondrial dysfunction in spinal muscular atrophy.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . MGI: J:164444
- Sleigh JN; Gillingwater TH; Talbot K. 2011. The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . MGI: J:140332
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- 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.. In: . . 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.. In: . . MGI: J:148541
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Additional - Smn1tm1Msd related
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- 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . MGI: J:164456
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- Bordet T. 2009. Generation of an SMN2 transgene (line 11). In: . . MGI: J:144852
- Bordet T. 2009. Generation of an SMN2 transgene (line 46). In: . . 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.. In: . . 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.. In: . . MGI: J:154248
- Bowerman M; Beauvais A; Anderson CL; Kothary R. 2010. Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model.. In: . . 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.. In: . . MGI: J:210674
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- 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.. In: . . 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.. In: . . MGI: J:213599
- Butchbach ME; Edwards JD; Burghes AH. 2007. Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy.. In: . . 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.. In: . . MGI: J:156779
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- Dal Mas A; Rogalska ME; Bussani E; Pagani F. 2015. Improvement of SMN2 pre-mRNA processing mediated by exon-specific U1 small nuclear RNA.. In: . . MGI: J:230271
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- 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.. In: . . MGI: J:168716
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- 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.. In: . . MGI: J:231749
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- Genabai NK; Ahmad S; Zhang Z; Jiang X; Gabaldon CA; Gangwani L. 2015. Genetic inhibition of JNK3 ameliorates spinal muscular atrophy.. In: . . MGI: J:226840
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- 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.. In: . . MGI: J:201094
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- 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.. In: . . 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.. In: . . 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.. In: . . 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.. In: . . MGI: J:208449
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