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
1 46,XY sex reversal 1 
Autosomal dominant, Autosomal recessive, X-linked dominant, Y-linked
Reproductive 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
2 Achalasia cardia 
Autosomal recessive
Gastrointestinal disorders NOS1 nitric oxide synthase 1
VIP vasoactive intestinal peptide
AAAS aladin WD repeat nucleoporin
CRLF1 cytokine receptor like factor 1
Reports
Updated as of Mar 09, 2023
PubMed
3 Achondrogenesis 
Autosomal dominant, Autosomal recessive
Bone disorders SLC26A2 solute carrier family 26 member 2
TRIP11 thyroid hormone receptor interactor 11
COL2A1 collagen type II alpha 1 chain
Reports
Updated as of Nov 30, 2023
PubMed
4 Acrodysostosis 
Autosomal dominant
Bone disorders PRKAR1A protein kinase cAMP-dependent type I regulatory subunit alpha
PDE4D phosphodiesterase 4D
Reports
Updated as of Feb 01, 2024
PubMed
5 Acromesomelic dysplasia 
Autosomal recessive
Bone disorders BMPR1B bone morphogenetic protein receptor type 1B
GDF5 growth differentiation factor 5
NPR2 natriuretic peptide receptor 2
ANK2 ankyrin 2
PRKG2 protein kinase cGMP-dependent 2
Reports
Updated as of Mar 09, 2023
PubMed
6 Acute intermittent porphyria 
Autosomal dominant
Metabolic disorders HMBS hydroxymethylbilane synthase
ABCB6 ATP binding cassette subfamily B member 6 (LAN blood group)
Reports
Updated as of Feb 01, 2024
PubMed
7 Acute lymphoid leukemia 
Cancer disorders FLT3 fms related receptor tyrosine kinase 3
ETV6 ETS variant transcription factor 6
PAX5 paired box 5
IKZF1 IKAROS family zinc finger 1
CDKN2A cyclin dependent kinase inhibitor 2A
Reports
Updated as of Mar 09, 2023
PubMed
8 Acute megakaryoblastic leukemia 
Cancer disorders GATA1 GATA binding protein 1
TP53 tumor protein p53
JAK3 Janus kinase 3
JAK1 Janus kinase 1
SETD2 SET domain containing 2, histone lysine methyltransferase
RAD21 RAD21 cohesin complex component
CTCF CCCTC-binding factor
SUZ12 SUZ12 polycomb repressive complex 2 subunit
SRSF2 serine and arginine rich splicing factor 2
Reports
Updated as of Jul 20, 2024
PubMed
9 Acute promyelocytic leukemia 
Blood disorders RARA retinoic acid receptor alpha
NUMA1 nuclear mitotic apparatus protein 1
Reports
Updated as of Jan 04, 2024
PubMed
10 Adisons disease 
Autosomal recessive
Endocrine disorders PTPN22 protein tyrosine phosphatase non-receptor type 22
HLA-B major histocompatibility complex, class I, B
HLA-DRB1 major histocompatibility complex, class II, DR beta 1
Reports
Updated as of Mar 09, 2023
PubMed