title: The role of SRY in mammalian sex determination.
abstract: The gene SRY (sex determining region of the Y), located at the distal region of the short arm of the Y chromosome, is necessary for male sex determination in mammals. SRY initiates the cascade of steps necessary to form a testis from an undifferentiated gonad. The SRY gene encodes an HMG (High Mobility Group) protein which may act as a transcription factor by binding to double stranded DNA and then bending the DNA. Mutations in SRY have been identified in some subjects with 46,XY pure gonadal dysgenesis. However the role for other autosomal and X-linked genes in testis determination is evident by the presence of a normal SRY gene in the majority of females with 46,XY pure gonadal dysgenesis and the lack of SRY in a minority of males with 46,XY maleness.
authors.affiliation: Division of Pediatric Endocrinology, Johns Hopkins University School of Medicine, Baltimore, MD 21287-3311, USA.
authors.forename: P Y
authors.initials: PY
authors.lastname: Fechner
mesh_terms: D000818:Animals; D004268:DNA-Binding Proteins / Q000235:genetics*; D012734:Disorders of Sex Development / Q000235:genetics; D005260:Female; D005801:Genes, Homeobox / Q000502:physiology; D006059:Gonadal Dysgenesis / Q000235:genetics; D006609:High Mobility Group Proteins / Q000235:genetics; D006801:Humans; D008297:Male; D009154:Mutation; D009687:Nuclear Proteins; D012732:Sex Determination Analysis; D012733:Sex Differentiation / Q000502:physiology*; D051876:Sex-Determining Region Y Protein; D014157:Transcription Factors; D014998:Y Chromosome / Q000235:genetics*
chemical_list: D004268:DNA-Binding Proteins; D006609:High Mobility Group Proteins; D009687:Nuclear Proteins; C497745:SRY protein, human; D051876:Sex-Determining Region Y Protein; D014157:Transcription Factors