GenTIGSA Gene Database on Rare Genetic Disorders

Monogenic disorders list  

List of monogenic disorders and their associated genes
Monogenic disorders arise from mutations in a single gene, but variations within that gene or in multiple genes can also lead to similar phenotypes, typically following Mendelian inheritance patterns associated with specific genetic changes.

View information by inheritance mode


S.No.  Genetic Disorders
Modes of inheritance
Category Gene symbol Indian
context
 Links
261 Toxic epidermal necrolysis 
Skin disorders HLA-B major histocompatibility complex, class I, B
IKZF1 IKAROS family zinc finger 1
Reports
Updated as of Sep 05, 2023
PubMed
262 Trichothiodystrophy 
Autosomal recessive
Skin disorders MPLKIP M-phase specific PLK1 interacting protein
GTF2H5 general transcription factor IIH subunit 5
ERCC3 ERCC excision repair 3, TFIIH core complex helicase subunit
ERCC2 ERCC excision repair 2, TFIIH core complex helicase subunit
RNF113A ring finger protein 113A
GTF2E2 general transcription factor IIE subunit 2
TARS1 threonyl-tRNA synthetase 1
AARS1 alanyl-tRNA synthetase 1
Reports
Updated as of Dec 29, 2023
PubMed
263 Tuberous Sclerosis 
Autosomal dominant
Tumor/Cancer TSC1 TSC complex subunit 1
TSC2 TSC complex subunit 2
SERPINC1 serpin family C member 1
Reports
Updated as of Sep 05, 2023
PubMed
264 Tyrosinemia type II 
Autosomal recessive
Metabolic disorders TAT tyrosine aminotransferase
HPD 4-hydroxyphenylpyruvate dioxygenase
Reports
Updated as of Jul 20, 2024
PubMed
265 Usher syndrome 
Autosomal recessive
Eye disorders / Ear disorders USH2A usherin
WHRN whirlin
USH1G USH1 protein network component sans
CLRN1 clarin 1
CDH23 cadherin related 23
PCDH15 protocadherin related 15
USH1C USH1 protein network component harmonin
ADGRV1 adhesion G protein-coupled receptor V1
MYO7A myosin VIIA
HARS1 histidyl-tRNA synthetase 1
PDZD7 PDZ domain containing 7
CIB2 calcium and integrin binding family member 2
PROM1 prominin 1
ESPN espin
ARSG arylsulfatase G
SLC9B1 solute carrier family 9 member B1
Reports
Updated as of Mar 09, 2023
PubMed
266 Uveal melanoma 
Autosomal dominant
Cancer disorders GNAQ G protein subunit alpha q
BAP1 BRCA1 associated protein 1
Reports
Updated as of Dec 29, 2023
PubMed
267 Van der Woude syndrome 1 
Autosomal dominant
Oral disorders IRF6 interferon regulatory factor 6
GRHL3 grainyhead like transcription factor 3
Reports
Updated as of Nov 02, 2023
PubMed
268 Very long chain acyl-CoA dehydrogenase deficiency 
Autosomal recessive
Metabolic disorders ACADVL acyl-CoA dehydrogenase very long chain
ACADL acyl-CoA dehydrogenase long chain
Reports
Updated as of Feb 01, 2025
PubMed
269 Vitiligo 
Skin disorders PTPN2 protein tyrosine phosphatase non-receptor type 2
NLRP1 NLR family pyrin domain containing 1
MTHFR methylenetetrahydrofolate reductase
Reports
Updated as of Sep 05, 2023
PubMed
270 Waardenburg syndrome 
Autosomal dominant
Eye disorders / Ear disorders PAX3 paired box 3
SOX10 SRY-box transcription factor 10
MITF melanocyte inducing transcription factor
EDNRB endothelin receptor type B
EDN3 endothelin 3
KITLG KIT ligand
Reports
Updated as of Sep 05, 2023
PubMed