B6.ROP/Le-Os/J

Stock number: 003523
Product name: oligosyndactylism
Research areas: Developmental Biology Research, Internal/Organ Research

Description

Mice carrying the radiation-induced oligosyndactyly (Os) mutation on a C57BL/6 genetic background exhibit hyperlipidemia, but do not develop glomerulosclerosis as do mice carrying Os on the ROP/GnLe strain.

Of note, the Os mutation is available on the FVB background (Stock No. MMRRC 067448) and the ROP background (Stock No. 000267).

Detailed description

Mice carrying the radiation-induced oligosyndactyly (Os) mutation on a C57BL/6 genetic background exhibit hyperlipidemia , but do not develop glomerulosclerosis as do mice carrying Os on the ROP/GnLe strain (Stock No. 000267). Nephron number is decreased by 50% in both B6 Os/+ and ROP Os/+ mice, and a glomerular volume and labeling index are two- to threefold increased in both. Chow-fed B6.ROP Os/+ animals demonstrate glomerular hypertrophy with an increase in mesangial matrix and cellularity that is characterized by macrophage influx and increased proliferation. Hyperlipidemia in B6.ROP Os/+ mice results in increased plasma BUN compared to chow-fed B6.ROP Os/+ animals and aggravated renal pathology by further increasing glomerular matrix and glomerular hypercellularity. Glomerular transforming growth factor-beta (TGF-beta) mRNA expression is increased in B6.ROP Os/+ mice compared to controls.

Development

The Os mutation was transferred from mice of strain ROP/Le onto the C57BL/6J background by repeated backcrossing reaching N27 in 2005.

Allele

Symbol: Os
Name: Os
MGI accession ID: MGI:1857646
Generation method: Radiation induced
Marker symbol: Os
Marker name: oligosyndactylism
Chromosome: 8
Strain of origin: (101 x C3H)F1
Molecular note: The oligosyndactylism mutation is due to a chromosomal inversion that has breakpoints approximately 10 Mb apart. One breakpoint appears to reside in the Anapc10 gene, and an aberrant transcript consisting of part of Anapc10 and an unrelated sequence is expressed at low levels.
General note: Heterozygotes are affected on all four feet. Fusion usually occurs between the second and third digits and occasionally involves the fourth (J:13049). The muscles of the forearms and lower legs as well as of the feet show anomalous arrangements not necessarily correlated with the skeletal changes (J:12944). At 11 days of gestation the preaxial border of the limbs can be seen to be reduced (J:12942), and a histological examination at this time shows that there is a small amount of cellular degeneration in the preaxial part of the footplate mesoderm, leading to coalescence of the second and third digital rudiments (J:5107). Os /+ mice have a mild diabetes insipidus present at 5 weeks and increasing with age. In combination with one or more recessive modifying genes in the selected DI stock, Os/+ mice have a severe diabetes insipidus (J:12948). The cause of the diabetes is a 45% reduction in size of the kidneys with an 80% reduction in number of glomeruli. Compensatory hypertrophy of the nephrons is not sufficient to restore normal urine-concentrating ability (J:5127)(J:5128).