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
281 Hyperekplexia 3 
Autosomal dominant, Autosomal recessive
Neurodevelopmental disorders SLC6A5 solute carrier family 6 member 5
Reports
Updated as of Feb 10, 2026
PubMed
282 Hypereosinophilic syndrome, idiopathic 
Somatic mutation
Blood disorders PDGFRA platelet derived growth factor receptor alpha
Reports
Updated as of Feb 20, 2024
PubMed
283 Hyperimmunoglobulin D with periodic fever 
Autosomal recessive
Immune disorders MVK mevalonate kinase
Reports
Updated as of Jan 05, 2026
PubMed
284 Hyperinsulinemic hypoglycemia, familial, 1 
Autosomal dominant, Autosomal recessive
Metabolic disorders ABCC8 ATP binding cassette subfamily C member 8
Reports
Updated as of Sep 08, 2025
PubMed
285 Hyperinsulinemic hypoglycemia, familial, 4 
Autosomal recessive
Metabolic disorders HADH hydroxyacyl-CoA dehydrogenase
Reports
Updated as of Sep 08, 2025
PubMed
286 Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome 
Autosomal recessive
Metabolic disorders SLC25A15 solute carrier family 25 member 15
Reports
Updated as of Jul 20, 2024
PubMed
287 Hyperphenylalaninemia due to tetrahydrobiopterin deficiency 
Autosomal recessive
Metabolic disorders GCH1 GTP cyclohydrolase 1
Reports
Updated as of Nov 30, 2023
PubMed
288 Hyperprolinemia type 2 
Autosomal recessive
Metabolic disorders ALDH4A1 aldehyde dehydrogenase 4 family member A1
Reports
Updated as of Feb 20, 2024
PubMed
289 Hypertrophic cardiomyopathy 14 
Autosomal dominant
Cardiovascular disorders MYH6 myosin heavy chain 6
Reports
Updated as of Mar 30, 2026
PubMed
290 Hypochondroplasia 
Autosomal dominant
Bone disorders FGFR3 fibroblast growth factor receptor 3
Reports
Updated as of Jan 05, 2026
PubMed