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forkhead box protein L2; forkhead transcription factor FOXL2; pituitary forkhead factor mouse homolog (FOXL2)

Function: probable transcriptional regulator Structure: contains 1 fork-head DNA-binding domain Compartment: nucleus Expression: - expressed in developing eyelid - transcribed very early in somatic cells of the developing gonad (before sex determination) - expression persists in the follicular cells of the adult ovary Pathology: - defects in FOXL2 are a cause of blepharophimosis syndrome - defects in FOXL2 are a cause of premature ovarian failure 3 - 402C->G point mutation in the FOXL2 gene may be associated with ovarian cancer (adult granulosa-cell tumors) [5] Genetics: - mutational hotspot in region coding for the poly-Ala domain, since 30% of all mutations in the ORF lead to poly-Ala expansions, resulting mainly in blepharophimosis syndrome (BPES) type 2

General

forkhead family transcription factor

Properties

SIZE: MW = 39 kD entity length = 376 aa COMPARTMENT: cell nucleus MOTIF: glycine residue {35-43} (9) fork head domain NAME: fork head domain SITE: 54-148 alanine residue {221-234} (14) proline residue {284-292} (9) alanine residue {301-304} (4)

Database Correlations

OMIM correlations MORBIDMAP 605597 UniProt P58012 Entrez Gene 668

References

  1. UniProt :accession P58012
  2. Entrez Gene :accession 668
  3. Human FOXL2 mutation database http://medgen.ugent.be/LOVD/index.php?select_db=FOXL2
  4. NAME=GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=FOXL2
  5. Shah SP et al Mutation of FOXL2 in granulosa-cell tumors of the ovary. N Engl J Med 2009 Jun 25; 360:2719. PMID: 19516027 - Shendure J and Stewart CJ. Cancer genomes on a shoestring budget. N Engl J Med 2009 Jun 25; 360:2781. PMID: 19516026