Overview

Also Known As:situs inversus viscerum

These mice carry a spontaneous mutation at the Dnah11 locus characterized by inverse placement of the visceral and thoracic organs and in anomalous positioning and interactions of blood vessels. They are suitable for use in applications related to the study of abnormal laterality in humans.

Genetic overview

Genetic Background	Generation

Dnah11^iv

Allele Type	Gene Symbol	Gene Name
Spontaneous	Dnah11	dynein, axonemal, heavy chain 11

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Research Applications

Developmental Biology Research

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Details

Detailed Description

DNAH11 is important for developmental control of organ positioning in the left-right axis such that homozygosity for the situs inversus viscerum (iv) mutant allele can result not only in inverse placement of the visceral and thoracic organs, but also in anomalous positioning and interactions of blood vessels (including the hepatic portal, inferior vena cava, and azygos vein) and modified shape of organs and blood vessels, including abnormal lobation of lungs or liver. Approximately 50% of mice homozygous for Dnah11^iv have situs inversus, and the likelihood of situs inversus is not impacted by whether the homozygous parent has situs inversus. This indicates that wild type Dnah11 instructs left-right asymmetry, and in the absence of functional Dnah11 the direction of this asymmetry is random. Heterotaxia is found in less than half of homozygotes and occurs equally in those that do and do not have situs inversus. While heterotaxia may be impacted by genetic background, the incidence of situs inversus has not shown this variation. Situs inversus can be identified shortly after birth, until the skin thickens at approximately day 5, by viewing the location of the milk-filled stomach through the skin. Homozygotes are generally viable and do breed, although poorreproductive performance with a high rate of resorption has been reported by Brown et al. (Development 1989). Some premature death has been reported and may be caused by deformities of the cardiac loop. (Hummel and Chapman, 1959; Layton 1976; Brown et al., 1989; Icardo and Colvee, 2001.)

Development

In 1956 Katherine Hummel reported finding situs inversus viscerum (iv) in 6 out of 42 mice in the F3 generation from a cross of a C3H/e female with an my/my male. This my/my male was likely from the line that was then being inbred to become My/Hu (see stock#000265). The my mutation was bred out of this new mutant stock and in 1972 the iv-bearing stock was transferred from Katherine Hummel to Robert Collins, both at The Jackson Laboratory. Collins began inbreeding from the outbred stock in 1975 and this generated the strain SI/Col (see stock#001045) which is homozygous for Dnah11^iv, a, and Tyrp1^b. SI/Col reached F62 in 1994. At some point the Dnah11^iv mutation was bred onto 129/Sv-ter but the details of this breeding have been lost. In 1981 males homozygous for Dnah11^iv on the 129/Sv-ter background were bred to C57BL/6J females to generate embryos for cryopreservation.

Genetics

Dnah11^iv

Allele Symbol: Dnah11^iv

Allele Name	situs inversus viscerum
Allele Type	Spontaneous
Allele Synonym(s)	iv
Gene Symbol and Name	Dnah11, dynein, axonemal, heavy chain 11
Gene Synonym(s)
Strain of Origin	(C3HeB/Fe x STOCK Frem2^my)F3
Chromosome	12
Molecular Note	A G-to-A transition mutation led to a substitution of glutamate with lysine at position 2271 (p.E2271K)in the encoded protein. This residue is located between the second and third P-loop motifs, a highly conserved region that constitutes the motor domain.

Disease/Phenotype

Disease Terms

Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).

primary ciliary dyskinesia 7

Research Areas By Phenotype

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

Genotype: Dnah11^iv related

Developmental Biology Research
- Internal/Organ Defects
  - heart: vasculature
  - situs inversus
  - heart

Mammalian Phenotype Terms by Genotype

The following phenotype information is associated with a similar, but not exact match to this JAX^® Mice strain

Genotype: Dnah11^iv/Dnah11^iv
involves: C3H

cellular phenotype

abnormal motile primary cilium morphology
- the nodal cilia are rigid and appear frozen

absent nodal flow
- nodal cilia seldom move and beads added to the extraembryonic fluid fail to display any net directional movement indicating a lack of nodal flow

embryo phenotype

abnormal motile primary cilium morphology
- the nodal cilia are rigid and appear frozen

absent nodal flow
- nodal cilia seldom move and beads added to the extraembryonic fluid fail to display any net directional movement indicating a lack of nodal flow

growth/size/body region phenotype

heterotaxia
- malous positioning of the hepatic portal vein, flatter more elongated spleen, and diminished or altered lobulation of the liver or lung
- situs inversus with incomplete penetrance and variable expressivity can yield complete situs inversus of thoracic and abdominal viscera and associated blood vessels, incomplete situs inversus, anomalous relationship of the postcaval and azygous veins, anomalous positioning of the hepatic portal vein, flatter more elongated spleen, and diminished or altered lobulation of the liver or lung

situs inversus
- about 50 per cent of homozygotes showed left-right transposition of stomach and abdominal viscera
- about 50% of all the homozygotes showed discordance in asymmetry between the stomach and the major abdominal and thoracic veins, the discordance occurring with similar frequency in mice with normal and reversed viscera
- Background Sensitivity - penetrance is 71%

Genotype: Dnah11^iv/Dnah11^iv
SI/Col Tyrp1 Dnah11/J

cardiovascular system phenotype

abnormal cardiovascular system morphology
7.6% display cardiac lesions, which are more common in females (11.5%) than in males (3.1%) and in fetuses (16%) than in adults (3.6%)
(MGI Ref ID J:4058)
homozygotes exhibit various cardiovascular, spleen and liver defects that may or may not co-occur in the same mouse
(MGI Ref ID J:4058)

abnormal heart atrium auricular region morphology
50.2% exhibit mirror-image arrangement of the atrial appendages
(MGI Ref ID J:4058)
incidence of isomerism of the atrial appendages is significantly higher in females (7.4%) than in males (1.6%) and in fetuses (12%) than in adults (1.4%)
(MGI Ref ID J:4058)

abnormal heart atrium morphology
(MGI Ref ID J:4058)

abnormal hepatic portal vein morphology
26.6% exhibit a ventral portal vein, with the incidence of the ventral location significantly higher in females (33.1%) than in males (18.9%)
(MGI Ref ID J:4058)

abnormal inferior vena cava morphology
17.8% show an abnormal connection of the inferior caval vein, with the abnormal arrangement more common in fetuses (29.3%) than in adults (15%)
(MGI Ref ID J:4058)

left atrial isomerism
3.3% have left atrial isomerism
(MGI Ref ID J:4058)

right atrial isomerism
1.5% have right atrial isomerism
(MGI Ref ID J:4058)

growth/size/body region phenotype

left atrial isomerism
3.3% have left atrial isomerism
(MGI Ref ID J:4058)

left pulmonary isomerism
a minority of both situs inversus and situs solitus mice have three lung lobes on the right, three lung lobes on the left, and one or two caudate lobes, instead of the normal three lobes on the right, one lobe on the left and one caudate lobe
(MGI Ref ID J:148465)

right atrial isomerism
1.5% have right atrial isomerism
(MGI Ref ID J:4058)

right pulmonary isomerism
a minority of both situs inversus and situs solitus mice have only one lung lobe on the right, one on the left and either one or no caudate lobe, instead of the normal three lobes on the right, one lobe on the left and one caudate lobe
(MGI Ref ID J:148465)

situs inversus
(MGI Ref ID J:148465)
seen in about 50% of homozygotes
(MGI Ref ID J:4058)

hematopoietic system phenotype

abnormal spleen morphology
36.4% exhibit an abnormal spleen, which is seen more often in females (44.6%) than in males (26.8%)
(MGI Ref ID J:4058)
spleen abnormalities include fissured, bilobed, elongated or absent spleen
(MGI Ref ID J:4058)

homeostasis/metabolism phenotype

increased physiological sensitivity to xenobiotic
treatment with acetazolamide on day 10 of pregnancy results in an increased rate of resorption, decreased fetal weight, and increased limb defects at embryonic day 19 compared with MF1 outbred mice
(MGI Ref ID J:148465)

immune system phenotype

abnormal spleen morphology
36.4% exhibit an abnormal spleen, which is seen more often in females (44.6%) than in males (26.8%)
(MGI Ref ID J:4058)
spleen abnormalities include fissured, bilobed, elongated or absent spleen
(MGI Ref ID J:4058)

limbs/digits/tail phenotype

abnormal autopod morphology
where most defects are found in normal fetuses exposed to these drugs
(MGI Ref ID J:148465)
culturing day 9 fetuses in misonidazole for 48 hours results in limb-bud abnormalities
(MGI Ref ID J:148465)
he loss of the ulna of the affected forearm
(MGI Ref ID J:148465)
these forelimb birth defects induced by acetazolamide or misonidazole follow the situs of the developing embryo such that those with situs inversus have defects in the left forelimb and those with situs solitus have defects in the right forelimb, which is where most defects are found in normal fetuses exposed to these drugs
(MGI Ref ID J:148465)
treatment of the pregnant dam with acetazolamide results in forelimb abnormalities, most commonly (66% of defects) the absence of the fifth digit, in more severe cases (11% of defects) the absence of the third, forth, and fifth digits, and in rare cases the loss of the ulna of the affected forearm
(MGI Ref ID J:148465)

liver/biliary system phenotype

abnormal liver morphology
a missing liver lobe and associated abnormal path of the inferior vena cava is found in a minority of both situs solitus and situs inversus mice, with the missing lobe usually on the right side in situs solitus and the left side in situs inversus
(MGI Ref ID J:148465)
abnormal arrangement of the liver, which is more common in females (42.6%) than in males (26.8%)
(MGI Ref ID J:4058)

reproductive system phenotype

abnormal embryo implantation
females with bilobed or fissured spleens or with abnormal livers have fewer implantation sites than those with normal spleens and livers
(MGI Ref ID J:4058)

reduced fertility
only 58% of dams with copulatory plugs are pregnant 19 days post-copulation
(MGI Ref ID J:148465)

respiratory system phenotype

left pulmonary isomerism
a minority of both situs inversus and situs solitus mice have three lung lobes on the right, three lung lobes on the left, and one or two caudate lobes, instead of the normal three lobes on the right, one lobe on the left and one caudate lobe
(MGI Ref ID J:148465)

right pulmonary isomerism
a minority of both situs inversus and situs solitus mice have only one lung lobe on the right, one on the left and either one or no caudate lobe, instead of the normal three lobes on the right, one lobe on the left and one caudate lobe
(MGI Ref ID J:148465)

References

Additional References
Icardo JM; Colvee E. 2001. Origin and course of the coronary arteries in normal mice and in iv/iv mice. J Anat 199(Pt 4):473-82PubMed: 11693308 MGI: J:73207
Layton WM Jr. 1976. Random determination of a developmental process: reversal of normal visceral asymmetry in the mouse. J Hered 67(6):336-8PubMed: 1021593 MGI: J:5788
Oh SP; Li E. 2002. Gene-dosage-sensitive genetic interactions between inversus viscerum (iv), nodal, and activin type IIB receptor (ActRIIB) genes in asymmetrical patterning of the visceral organs along the left-right axis. Dev Dyn 224(3):279-90PubMed: 12112458 MGI: J:78247
Supp DM; Witte DP; Potter SS; Brueckner M. 1997. Mutation of an axonemal dynein affects left-right asymmetry in inversus viscerum mice. Nature 389(6654):963-6PubMed: 9353118 MGI: J:44093

Additional - Dnah11^iv related

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When using the situs inversus viscerum mouse strain in a publication, please cite the originating article(s) and include JAX stock #000773 in your Materials and Methods section.

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Heterozygous for Dnahc11<iv>

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Also Known As:situs inversus viscerum

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Dnah11iv

Allele Symbol: Dnah11iv

Disease Terms

Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).

Research Areas By Phenotype

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

Genotype: Dnah11iv related

Mammalian Phenotype Terms by Genotype

Genotype: Dnah11iv/Dnah11ivinvolves: C3H

cellular phenotype

embryo phenotype

growth/size/body region phenotype

Genotype: Dnah11iv/Dnah11ivSI/Col Tyrp1 Dnah11/J

cardiovascular system phenotype

growth/size/body region phenotype

hematopoietic system phenotype

homeostasis/metabolism phenotype

immune system phenotype

limbs/digits/tail phenotype

liver/biliary system phenotype

reproductive system phenotype

respiratory system phenotype

References

Additional References

Additional - Dnah11iv related

Genotyping Protocols

Citation

Animal Health Reports

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

Live Mouse

Cryorecovery - Pricing

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms Of Use

Licensing Information

JAX® Mice, Products & Services Conditions of Use

No Warranty

Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Dnah11^iv

Allele Symbol: Dnah11^iv

Genotype: Dnah11^iv related

Genotype: Dnah11^iv/Dnah11^iv
involves: C3H

Genotype: Dnah11^iv/Dnah11^iv
SI/Col Tyrp1 Dnah11/J

Additional - Dnah11^iv related