GenTIGSA Gene Database on Rare Genetic Disorders

Select a RGD Category for details 

RGDs registry under the 'Reproductive disorders'
Disorder Name (Total=7) Aliases Sub-Category Gene Indian Reports Link
3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency
Autosomal recessive
•Steroid 5-alpha-reductase 2 deficiency
•46,XY DSD due to 5-alpha-reductase 2 deficiency
• Pseudovaginal perineoscrotal hypospadias
•Familial incomplete male pseudohermaphroditism, type 2
•Male pseudohermaphroditism due to 5-alpha-reductase deficiency
Gonadal disorders SRD5A2 steroid 5 alpha-reductase 2
Reports
Updated as of Jul 20, 2024
PubMed
46,XY sex reversal 1
Autosomal dominant, Autosomal recessive, X-linked dominant, Y-linked
•Swyer syndrome
•46,XY complete gonadal dysgenesis
•Pure gonadal dysgenesis 46,XY
•46, XY CGD
•46, XY pure gonadal dysgenesis
•46,XY sex reversal
•Gonadal dysgenesis, XY female type
Sexual differenciation disorders SRY sex determining region Y
SOX9 SRY-box transcription factor 9
DHX37 DEAH-box helicase 37
Reports
Updated as of Jul 20, 2024
PubMed
Hydatidiform mole, recurrent, 1
Autosomal recessive
•Hydatidiform mole
•HYDM1
•Hydatidiform moles
•Hydatidiform mole, recurrent
•Gestational trophoblastic disease
•Complete hydatidiform mole
•Hydatidiform mole, complete
•Classical hydatidiform mole
•Complete molar pregnancy
•Hydatidiform moles
•Molar pregnancy
Placenta neoplasm NLRP7 NLR family pyrin domain containing 7
Reports
Updated as of Mar 30, 2026
PubMed
Mayer Rokitansky Kuster Hauser syndrome type 1
Autosomal dominant
•MRKH syndrome type 1
•Mayer Rokitansky Küster hauser syndrome type 1
•Mayer-Rokitansky-Kuster-hauser syndrome type 1
•Mayer-rokitansky-Küster-Hauser syndrome type 1
•Mayer Rokitansky Küster Hauser syndrome type 1
•MRKH (Mayer Rokitansky Küster Hauser) syndrome type 1
•MRKH
GREB1L GREB1 like retinoic acid receptor coactivator
Reports
Updated as of Aug 17, 2026
PubMed
Mayer-Rokitansky-Küster-Hauser syndrome type 2
Autosomal dominant
•Klippel-Feil deformity, conductive deafness, and absent vagina
•Atypical mrkh (mayer rokitansky kuster hauser) syndrome
•Atypical mrkh syndrome
•Mayer-rokitansky-kuster-hauser syndrome, type ii
• mrkh syndrome type 2
•MRKH, type ii
•Mullerian duct aplasia-renal dysplasia-cervical somite anomalies syndrome
•Mullerian duct aplasia, renal dysplasia, cervical somite anomalies syndrome
•Mullerian duct aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies
•Murcs association
•Müllerian duct aplasia-renal dysplasia-cervical somite anomalies syndrome
- GREB1L GREB1 like retinoic acid receptor coactivator
Reports
Updated as of Aug 17, 2026
PubMed
Chondrodysplasia-pseudohermaphroditism syndrome
Autosomal recessive
•Nivelon Nivelon Mabille syndrome
•Chondrodysplasia pseudohermaphrodism syndrome
•Chondrodysplasia pseudohermaphroditism syndrome
•Chondrodysplasia with disorder of sex development syndrome
•Chondrodysplasia-disorder of sex development syndrome
•Nivelon-nivelon-mabille syndrome
•Pseudohermaphrodism and chondrodysplasia
Gonadal disorders HHAT hedgehog acyltransferase
Reports
Updated as of Aug 17, 2026
PubMed
46,XY sex reversal 3
Autosomal dominant
•46,xy gonadal dysgenesis, partial or complete, with or without adrenal failure
•46,xy sex reversal, partial or complete, nr5a1-related
•Disorder of sex development, 46,xy, nr5a1-related
•NR5AL-related 46,xy complete gonadal dysgenesis
•Sex reversal, xy, with or without adrenal failure
•SRXY3
Sexual differenciation disorders NR5A1 nuclear receptor subfamily 5 group A member 1
GATA4 GATA binding protein 4
Reports
Updated as of Aug 17, 2026
PubMed