FAM58A

General Information

Full gene name:family with sequence similarity 58, member A
Entrez Gene ID:92002
Location:Xq28
Synonyms:STAR
Type:protein-coding

User SNPs

SNPs given by the user that are near or inside this gene:

SNP Distance (bp) Direction
rs3020789 28355 upstream

NCBI Summary

Mutations in this gene have been shown to cause an X-linked dominant STAR syndrome that typically manifests syndactyly, telecanthus and anogenital and renal malformations. The protein encoded by this gene contains a cyclin-box-fold domain which suggests it may have a role in controlling nuclear cell division cycles. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Oct 2008]

OMIM

OMIM ID:`OMIM ID 300708 `_

Allelic Variants (Selected Examples)

.0001 STAR SYNDROME

In a girl with STAR syndrome (300707) and additional features of lower lid coloboma, epilepsy, and syringomyelia, Unger et al. (2008) found a heterozygous deletion on chromosome Xq28 of approximately 40 kb that removed exons 1 and 2 as well as intron 1, a portion of intron 2, and the 5-prime untranslated region, of the FAM58A gene.

.0002 STAR SYNDROME

In a girl with STAR syndrome (300707), Unger et al. (2008) detected a de novo heterozygous deletion of 4,249 bp which removed 1,265 bp of intron 4, all of exon 5 including the 3-prime untranslated region, and 2,454 bp of 3-prime sequence.

.0003 STAR SYNDROME

In a patient with STAR syndrome (300707), Unger et al. (2008) identified a mutation in the FAM58A gene affecting the splice donor site of intron 4: 555+1G-A.

.0004 STAR SYNDROME

In a girl with STAR syndrome (300707), Unger et al. (2008) identified a frameshift mutation, 201dupT, in the FAM58A gene, which immediately resulted in a premature stop codon (N68XfsX1).

.0005 STAR SYNDROME

In the mother and daughter reported by Green et al. (1996) with STAR syndrome (300707), Unger et al. (2008) found a mutation in the FAM58A gene, 556-1G-A, which altered the splice acceptor site of intron 4.

NCBI Phenotypes

  • STAR syndrome
  • Gene Reviews
  • GTR
  • OMIM

Gene Ontology

  • protein binding
  • regulation of cyclin-dependent protein kinase activity
  • protein kinase binding
  • regulation of transcription, DNA-dependent

KEGG Pathways

No pathways found linked to this gene.

GeneRIFs

  • Mutations in the cyclin family member FAM58A cause an X-linked dominant disorder characterized by syndactyly, telecanthus and anogenital and renal malformations. [PMID 18297069]

PubMed Articles

Recent articles:

  • Unger S et al. “Mutations in the cyclin family member FAM58A cause an X-linked dominant disorder characterized by syndactyly, telecanthus and anogenital and renal malformations.” Nat Genet. 2008 Mar;40(3):287-9. PMID 18297069
  • Kim JM et al. “Identification of genes related to Parkinson’s disease using expressed sequence tags.” DNA Res. 2006 Dec 31;13(6):275-86. PMID 17213182
  • Mehrle A et al. “The LIFEdb database in 2006.” Nucleic Acids Res. 2006 Jan 1;34(Database issue):D415-8. PMID 16381901
  • Wiemann S et al. “From ORFeome to biology: a functional genomics pipeline.” Genome Res. 2004 Oct;14(10B):2136-44. PMID 15489336
  • Strausberg RL et al. “Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.” Proc Natl Acad Sci U S A. 2002 Dec 24;99(26):16899-903. PMID 12477932
  • Hartley JL et al. “DNA cloning using in vitro site-specific recombination.” Genome Res. 2000 Nov;10(11):1788-95. PMID 11076863

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