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VARIABLY CHARGED, X CHROMOSOME; VCX

VARIABLY CHARGED, X CHROMOSOME; VCX

Alternative titles; symbolsVCX1VARIABLY CHARGED, X CHROMOSOME, WITH 10 REPEATS; VCX10RVARIABLY CHARGED, X CHROMOSOME, B1; VCXB1HGNC Approved Gene Symbol: VCXCyto...

Alternative titles; symbols

  • VCX1
  • VARIABLY CHARGED, X CHROMOSOME, WITH 10 REPEATS; VCX10R
  • VARIABLY CHARGED, X CHROMOSOME, B1; VCXB1

HGNC Approved Gene Symbol: VCX

Cytogenetic location: Xp22.31 Genomic coordinates (GRCh38): X:7,842,261-7,844,142 (from NCBI)

▼ Cloning and Expression
By screening a testis cDNA library using VCY (400012) as probe, Lahn and Page (2000) obtained 3 novel VCY-like sequences, including VCX, which they designated VCX10R. The 747-nucleotide VCX10R transcript differs from VCY and other VCX transcripts primarily in the number of copies of a 30-nucleotide sequence. VCY contains a single copy, whereas VCX10R contains 10 repeats and other VCX transcripts contain at least 2 repeats. Outside the repeat region, the open reading frames of the cDNAs share at least 96% nucleotide identity. The 10-amino acid motif encoded by the repeat is rich in glutamic acid and is predicted to be highly negatively charged. Outside the repeat regions, VCY and VCX proteins are predicted to be highly positively charged. Therefore, the number of repeats within a given protein is likely to alter its overall charge. The deduced VCX proteins also contain an N-terminal bipartite nuclear localization signal. RT-PCR of several tissues detected VCY and VCX transcripts only in testis, but not in testis lacking germ cells (Sertoli cells only).

▼ Gene Structure
Lahn and Page (2000) determined that the coding region of the VCX10R gene contains 2 exons.

▼ Mapping
By radiation hybrid analysis, Lahn and Page (2000) mapped the VCX10R gene to a VCX cluster near the steroid sulfatase gene (STS; 300747) on chromosome Xp22.3. They also found that a copy of VCX containing 11 repeats is present in CRI-S232 (DXS278), a genomic fragment derived from the X chromosome. Aberrant recombination between arrays of CRI-S232-homologous repeats flanking the STS gene results in STS deletion, which is manifested clinically as X-linked ichthyosis. The findings of Lahn and Page (2000) that CRI-S232 contains a VCX gene offers a more precise description of the genetic etiology of X-linked ichthyosis: it results from aberrant recombination between VCX gene arrays that flank the STS locus.

Tags: XP22.31