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NPHP4

MIM.607215 1p36

nephroretinin, nephrocystin-4, KIAA0673
Locus: 1p36

Pathology

- mutation in juvenile nephronophthisis type 4 (NPHP4)
- mutation in juvenile nephronophthisis and retinitis pigmentosa association (Senior-Loken syndrome) (SLS) (locus NPHP4 in 1p36) (12205563)

See also

- RPGRIP1L (mutated in Joubert syndrome type B or CORS)

  • RPGRIP1L interacts with nephrocystin-4 and that mutations in the gene encoding nephrocystin-4 (NPHP4)

References

- Roepman R, Letteboer SJ, Arts HH, van Beersum SE, Lu X, Krieger E, Ferreira PA, Cremers FP. Interaction of nephrocystin-4 and RPGRIP1 is disrupted by nephronophthisis or Leber congenital amaurosis-associated mutations. Proc Natl Acad Sci U S A. 2005 Dec 20;102(51):18520-5. PMID: 16339905

- Hoefele J, Sudbrak R, Reinhardt R, Lehrack S, Hennig S, Imm A, Muerb U, Utsch B, Attanasio M, O?Toole JF, Otto E, Hildebrandt F. Mutational analysis of the NPHP4 gene in 250 patients with nephronophthisis. Hum Mutat. 2005 Apr;25(4):411. PMID: 15776426

- Mollet G, Salomon R, Gribouval O, Silbermann F, Bacq D, Landthaler G, Milford D, Nayir A, Rizzoni G, Antignac C, Saunier S. The gene mutated in juvenile nephronophthisis type 4 encodes a novel protein that interacts with nephrocystin. Nat Genet. 2002 Oct;32(2):300-5. PMID: 12244321

- Otto E, Hoefele J, Ruf R, Mueller AM, Hiller KS, Wolf MT, Schuermann MJ, Becker A, Birkenhager R, Sudbrak R, Hennies HC, Nurnberg P, Hildebrandt F. A gene mutated in nephronophthisis and retinitis pigmentosa encodes a novel protein, nephroretinin, conserved in evolution. Am J Hum Genet. 2002 Nov;71(5):1161-7. PMID: 12205563

- Schuermann MJ, Otto E, Becker A, Saar K, Ruschendorf F, Polak BC, Ala-Mello S, Hoefele J, Wiedensohler A, Haller M, Omran H, Nurnberg P, Hildebrandt F. Mapping of gene loci for nephronophthisis type 4 and Senior-Loken syndrome, to chromosome 1p36. Am J Hum Genet. 2002 May;70(5):1240-6. PMID: 11920287