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 3 beta-Hydroxysteroid dehydrogenase deficiency 
Autosomal recessive
Endocrine disorders HSD3B2 hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 2
Reports
Updated as of Sep 08, 2025
PubMed
2 3-hydroxy-3-methylglutaryl-CoA synthase deficiency 
Autosomal recessive
Metabolic disorders HMGCS2 3-hydroxy-3-methylglutaryl-CoA synthase 2
Reports
Updated as of Feb 20, 2024
PubMed
3 3-Methylglutaconic aciduria type 2 
X-linked dominant
Metabolic disorders TAFAZZIN tafazzin, phospholipid-lysophospholipid transacylase
Reports
Updated as of Jul 20, 2024
PubMed
4 3-Oxo-5 alpha-steroid delta 4-dehydrogenase deficiency 
Autosomal recessive
Reproductive disorders SRD5A2 steroid 5 alpha-reductase 2
Reports
Updated as of Jul 20, 2024
PubMed
5 8q24.3 microdeletion syndrome 
Autosomal dominant
Multisystemic disorders PUF60 poly(U) binding splicing factor 60
Reports
Updated as of Sep 08, 2025
PubMed
6 Abetalipoproteinaemia 
Autosomal recessive
Metabolic disorders MTTP microsomal triglyceride transfer protein
Reports
Updated as of Feb 01, 2024
PubMed
7 Achondroplasia 
Autosomal dominant
Bone disorders FGFR3 fibroblast growth factor receptor 3
Reports
Updated as of Nov 30, 2023
PubMed
8 Acrocallosal syndrome 
Autosomal recessive
Bone disorders KIF7 kinesin family member 7
Reports
Updated as of Nov 10, 2023
PubMed
9 Acrocephalosyndactyly 
Autosomal dominant
Bone disorders FGFR2 fibroblast growth factor receptor 2
Reports
Updated as of Sep 15, 2022
PubMed
10 Acrodermatitis continua suppurativa of Hallopeau 
Autosomal recessive
Skin disorders IL36RN interleukin 36 receptor antagonist
Reports
Updated as of Jul 20, 2024
PubMed