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Gene Review

Bicc1  -  bicaudal C homolog 1 (Drosophila)

Mus musculus

Synonyms: Bic-C, Protein bicaudal C homolog 1, bpk, jcpk
 
 
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Disease relevance of Bicc1

  • A chlorambucil (CHL)-induced mutation of the jcpk (juvenile congenital polycystic kidney disease) gene causes a severe early onset polycystic kidney disease [1].
  • The order and genetic distances of all markers and gene loci mapped in the jcpk intercross were consistent with those derived from the BALB/c backcross, indicating that the CHL-induced lesion has not generated any gross chromosomal abnormalities detectable in these studies [1].
  • Previously, we have described the mouse bpk mutation, a model that closely resembles human autosomal recessive polycystic kidney disease [2].
  • Treatment of cystic bpk mice with WTACE2 resulted in a 43% reduction in kidney weight to body weight ratio (11.2 vs. 19.7%), improved cystic index (3.2 vs. 4.8), reduced cystic CT to PT ratio (1.2 vs. 8), and a greater than 30% reduction in BUN and serum creatinine [3].
  • In X-linked agammaglobulinemia the molecular defect was found to reside in the gene encoding a novel cytoplasmic tyrosine kinase (bpk, atk, or btk) expressed by B and myeloid cells [4].
 

High impact information on Bicc1

 

Chemical compound and disease context of Bicc1

  • METHODS: Amphiregulin and heparin-binding-EGF expression were examined by immunohistology and Western blot of kidneys and conditionally-immortalized collecting tubule cells obtained from cystic bpk mice (a murine model of ARPKD) and normal littermates [9].
 

Biological context of Bicc1

  • It is concluded that the severity of the bpk renal cystic disease phenotype is modulated by multiple loci and possibly by epistatic interaction among them [8].
  • In the course of these studies, marked variability was observed in the renal cystic disease expressed in F2 bpk/bpk homozygotes of a (BALB/c-+/bpk x CAST/Ei)F1 intercross [8].
  • The jcpk intercross allowed the orientation of the Ank3 gene relative to the centromere to be determined [1].
  • In addition, approximately 25% of the aged +/jcpk heterozygotes show evidence of glomerulocystic disease [10].
  • Additionally, sixty-nine kidney-specific ESTs were evaluated as candidates for jcpk and subsequently localized throughout the mouse genome by radiation hybrid mapping analysis [11].
 

Anatomical context of Bicc1

 

Associations of Bicc1 with chemical compounds

  • In the homozygous condition, the gene, jcpk, causes a very severe disease characterized by cysts in all segments of the nephron [10].
 

Analytical, diagnostic and therapeutic context of Bicc1

  • In order to positionally clone this gene, high resolution genetic and radiation hybrid maps were generated along with a detailed physical map of the approximately 500-kb region containing the jcpk gene [11].
  • METHODS: Immunohistochemistry (IH) and Western analysis were performed on kidneys from cystic bpk mice and noncystic littermates at postnatal days 7, 14, and 21 [3].

References

  1. Fine genetic map of mouse chromosome 10 around the polycystic kidney disease gene, jcpk, and ankyrin 3. Bryda, E.C., Ling, H., Rathbun, D.E., Burmeister, M., Flaherty, L. Genomics (1996) [Pubmed]
  2. Evidence that two phenotypically distinct mouse PKD mutations, bpk and jcpk, are allelic. Guay-Woodford, L.M., Bryda, E.C., Christine, B., Lindsey, J.R., Collier, W.R., Avner, E.D., D'Eustachio, P., Flaherty, L. Kidney Int. (1996) [Pubmed]
  3. A novel inhibitor of tumor necrosis factor-alpha converting enzyme ameliorates polycystic kidney disease. Dell, K.M., Nemo, R., Sweeney, W.E., Levin, J.I., Frost, P., Avner, E.D. Kidney Int. (2001) [Pubmed]
  4. The molecular basis of X-linked agammaglobulinemia, hyper-IgM syndrome, and severe combined immunodeficiency in humans. Aruffo, A., Hollenbaugh, D., Wu, L.H., Ochs, H.D. Curr. Opin. Hematol. (1994) [Pubmed]
  5. Mutation of unique region of Bruton's tyrosine kinase in immunodeficient XID mice. Rawlings, D.J., Saffran, D.C., Tsukada, S., Largaespada, D.A., Grimaldi, J.C., Cohen, L., Mohr, R.N., Bazan, J.F., Howard, M., Copeland, N.G. Science (1993) [Pubmed]
  6. Identification of direct serum-response factor gene targets during Me2SO-induced P19 cardiac cell differentiation. Zhang, S.X., Garcia-Gras, E., Wycuff, D.R., Marriot, S.J., Kadeer, N., Yu, W., Olson, E.N., Garry, D.J., Parmacek, M.S., Schwartz, R.J. J. Biol. Chem. (2005) [Pubmed]
  7. Role of CFTR in autosomal recessive polycystic kidney disease. Nakanishi, K., Sweeney, W.E., Macrae Dell, K., Cotton, C.U., Avner, E.D. J. Am. Soc. Nephrol. (2001) [Pubmed]
  8. Quantitative trait loci modulate renal cystic disease severity in the mouse bpk model. Guay-Woodford, L.M., Wright, C.J., Walz, G., Churchill, G.A. J. Am. Soc. Nephrol. (2000) [Pubmed]
  9. EGF-related growth factors in the pathogenesis of murine ARPKD. MacRae Dell, K., Nemo, R., Sweeney, W.E., Avner, E.D. Kidney Int. (2004) [Pubmed]
  10. New mouse model for polycystic kidney disease with both recessive and dominant gene effects. Flaherty, L., Bryda, E.C., Collins, D., Rudofsky, U., Montogomery, J.C. Kidney Int. (1995) [Pubmed]
  11. Characterization of the region containing the jcpk PKD gene on mouse Chromosome 10. Price, S.J., Chittenden, L.R., Flaherty, L., O'Dell, B., Guay-Woodford, L.M., Stubbs, L., Bryda, E.C. Cytogenet. Genome Res. (2002) [Pubmed]
  12. Renal dysfunction but not cystic change is ameliorated by neonatal epidermal growth factor in bpk mice. Nakanishi, K., Gattone, V.H., Sweeney, W.E., Avner, E.D. Pediatr. Nephrol. (2001) [Pubmed]
 
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