Lpn1^NZB
QTL Variant Detail

Summary

• QTL variant: Lpn1^NZB
• Name: NZ lupus nephritis 1; NZB
• MGI ID: MGI:3621990
• QTL: Lpn1 Location: unknown Genetic Position: Chr17, Syntenic

Variant origin

• Strain of Specimen: NZB

Variant description

• Allele Type: QTL
• Mutation: Undefined
• This allele confers increased incidence of lupus nephritis compared to NZW. (J:6026)
• Inheritance: Dominant

Expression

In Structures Affected by this Mutation:

1 anatomical structure(s)

Notes

Mapping and Phenotype information for this QTL, its variants and associated markers

J:6026

Inbred strains NZB and NZW exhibit a low incidence of renal disease (8% and 2.5%, respectively). Interestingly, (NZB x NZW)F1 hybrid animals exhibit a high incidence of renal disease similar to human lupus nephritis (92% by 440 days of age). 150 (NZB x NZW)F1 x NZB backcross animals were analyzed for genetic linkage to renal disease.

High incidence of renal disease in backcross animals was associated with heterozygosity at the H2 locus on mouse Chromosome 17. An NZB-derived locus named Lpn1 (NZ lupus nephritis 1) showed loose linkage with the H2 locus at approximately 55 cM. An NZW-derived locus named Lpn2 (NZ lupus nephritis 2) is thought to be closely linked to the H2 locus. This was confirmed in 190 (NZB x NZW)F1 x NZC outcross animals where 10 outcross animals developed severe nephritis and were all heterozygous for the NZW-derived allele at the H2 locus. The immune response gene Ir1 may be a candidate for Lpn2.

A third locus named Lpn3 (NZ lupus nephritis 3) not linked to H2 is thought to account for the remainder of the high nephritis incidence in (NZB x NZW)F1 animals. Lpn3 is thought to be NZW-derived and has not been mapped.

References

• Original: J:6026 Knight JG, et al., Three genes for lupus nephritis in NZB x NZW mice. J Exp Med. 1978 Jun 1;147(6):1653-60
• All: 1 reference(s)