[The genetics of sex determination].
The male sex determination controls the testis determination and differentiation. So far more than a dozen of gene have been characterised, including the primary Y male sex determinant: SRY gene. All the genes encode transcription factor except FDF9 which encodes a growth factor allowing mesonephritic cell to divide and migrate to the mesenchyme. All these gene products are sensitive in man (but not in the mouse model) to gene dosage: haplo-insufficiency and or duplication of these genes all lead to sex reversal. Today, we still do not know how their products are epistatic in the sex determining cascade. So far no ovary sex determining gene has been characterised.
Vialard
François
F
Service d'Histologie, Embryologie, Cytogénétique, Biologie de la Reproduction et Génétique Médicale, CHI Poissy-Sant Germain, Poissy.
Fellous
Marc
M
D000818:Animals; D054304:Anti-Mullerian Hormone; D057137:DAX-1 Orphan Nuclear Receptor; D004268:DNA-Binding Proteins / Q000235:genetics / Q000502:physiology; D005260:Female; D051717:Fushi Tarazu Transcription Factors; D016162:Genes, Wilms Tumor; D024282:Genes, sry; D005835:Genitalia / Q000196:embryology; D006023:Glycoproteins; D006066:Gonads / Q000166:cytology / Q000196:embryology; D006131:Growth Inhibitors / Q000235:genetics / Q000502:physiology; D006609:High Mobility Group Proteins / Q000235:genetics / Q000502:physiology; D018398:Homeodomain Proteins; D006801:Humans; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D018160:Receptors, Cytoplasmic and Nuclear; D018168:Receptors, Retinoic Acid / Q000235:genetics / Q000502:physiology; D012097:Repressor Proteins; D055758:SOX9 Transcription Factor; D019849:Sex Determination Processes; D054339:Steroidogenic Factor 1; D013735:Testicular Hormones / Q000235:genetics / Q000502:physiology; D014157:Transcription Factors / Q000235:genetics / Q000502:physiology
D057137:DAX-1 Orphan Nuclear Receptor; D004268:DNA-Binding Proteins; D051717:Fushi Tarazu Transcription Factors; D006023:Glycoproteins; D006131:Growth Inhibitors; D006609:High Mobility Group Proteins; D018398:Homeodomain Proteins; C539965:NR0B1 protein, human; C539966:Nr0b1 protein, mouse; D018160:Receptors, Cytoplasmic and Nuclear; D018168:Receptors, Retinoic Acid; D012097:Repressor Proteins; D055758:SOX9 Transcription Factor; C527932:SOX9 protein, human; C527933:Sox9 protein, mouse; D054339:Steroidogenic Factor 1; D013735:Testicular Hormones; D014157:Transcription Factors; D054304:Anti-Mullerian Hormone