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Gene Review:

VCX3A - variable charge, X-linked 3A

Homo sapiens

Synonyms: VCX-8R, VCX-8r, VCX-A, VCX3, VCX8R, ...

Jiao, X. et al., Zou, S.W. et al., Fukami, M. et al., Lesca, G. et al., Dessay, S. et al., et al.

Hosomi, Oiso, Fukai, Hanada, Fujita, Ishii, Dessay, Moizard, Gilardi, Opitz, Middleton-Price, Pembrey, Moraine, Briault,

Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of VCX3A

Deletion of VCX-A due to NAHR plays a major role in the occurrence of mental retardation in patients with X-linked ichthyosis [1].
Furthermore, we demonstrate that a previously identified testis-specific protein of unknown function implicated in nonspecific X-linked mental retardation, VCX-A, can function as an inhibitor of hDcp2 decapping in vitro and in cells [2].

Psychiatry related information on VCX3A

A member of a gene family on Xp22.3, VCX-A, is deleted in patients with X-linked nonspecific mental retardation [3].

High impact information on VCX3A

Our data demonstrate that VCX-A can regulate mRNA stability and that it is an example of a tissue-specific decapping regulator [2].
Only one gene, VCX-A (variably charged, X chromosome mRNA on CRI-S232A), was shown to reside in this interval [3].
Xp22.3 microdeletion including VCX-A and VCX-B1 genes in an X-linked ichthyosis family: no difference in deletion size for patients with and without mental retardation [4].
In addition, the colocalization for VCY and VCX-8r in COS7 cells was also observed [5].
3. In this region, two potential candidate genes, VCX-A and PRKX, were excluded by sequence analysis of the coding region in patients of the two reported FG families [6].

Biological context of VCX3A

Deletions of VCX-A and NLGN4: a variable phenotype including normal intellect [7].

Associations of VCX3A with chemical compounds

Using propidium iodide staining and green fluorescent protein (GFP) tag technologies, VCY and VCX-8r proteins were mainly localized in the nucleoli of COS7 cells [5].

Other interactions of VCX3A

Recently, VCXA, which is located approximately 0.7Mb telomeric to the STS gene, was reported as a candidate gene for mental retardation (MR) in patients with XLI [8].
VCX-A deficiency has been shown previously to be associated with mental retardation and NLGN4 mutations lead to mental retardation in conjunction with autism [9].

References

Deletion of VCX-A due to NAHR plays a major role in the occurrence of mental retardation in patients with X-linked ichthyosis. Van Esch, H., Hollanders, K., Badisco, L., Melotte, C., Van Hummelen, P., Vermeesch, J.R., Devriendt, K., Fryns, J.P., Marynen, P., Froyen, G. Hum. Mol. Genet. (2005) [Pubmed]
Identification of an mRNA-Decapping Regulator Implicated in X-Linked Mental Retardation. Jiao, X., Wang, Z., Kiledjian, M. Mol. Cell (2006) [Pubmed]
A member of a gene family on Xp22.3, VCX-A, is deleted in patients with X-linked nonspecific mental retardation. Fukami, M., Kirsch, S., Schiller, S., Richter, A., Benes, V., Franco, B., Muroya, K., Rao, E., Merker, S., Niesler, B., Ballabio, A., Ansorge, W., Ogata, T., Rappold, G.A. Am. J. Hum. Genet. (2000) [Pubmed]
Xp22.3 microdeletion including VCX-A and VCX-B1 genes in an X-linked ichthyosis family: no difference in deletion size for patients with and without mental retardation. Lesca, G., Sinilnikova, O., Theuil, G., Blanc, J., Edery, P., Till, M. Clin. Genet. (2005) [Pubmed]
Expression and localization of VCX/Y proteins and their possible involvement in regulation of ribosome assembly during spermatogenesis. Zou, S.W., Zhang, J.C., Zhang, X.D., Miao, S.Y., Zong, S.D., Sheng, Q., Wang, L.F. Cell Res. (2003) [Pubmed]
FG syndrome: linkage analysis in two families supporting a new gene localization at Xp22.3 [FGS3]. Dessay, S., Moizard, M.P., Gilardi, J.L., Opitz, J.M., Middleton-Price, H., Pembrey, M., Moraine, C., Briault, S. Am. J. Med. Genet. (2002) [Pubmed]
Deletions of VCX-A and NLGN4: a variable phenotype including normal intellect. Macarov, M., Zeigler, M., Newman, J.P., Strich, D., Sury, V., Tennenbaum, A., Meiner, V. Journal of intellectual disability research : JIDR (2007) [Pubmed]
Deletion of distal promoter of VCXA in a patient with X-linked ichthyosis associated with borderline mental retardation. Hosomi, N., Oiso, N., Fukai, K., Hanada, K., Fujita, H., Ishii, M. J. Dermatol. Sci. (2007) [Pubmed]
Molecular cytogenetic analysis of a familial interstitial deletion Xp22.2-22.3 with a highly variable phenotype in female carriers. Chocholska, S., Rossier, E., Barbi, G., Kehrer-Sawatzki, H. Am. J. Med. Genet. A (2006) [Pubmed]

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