GenTIGSA Gene Database on Rare Genetic Disorders
  Beta Version

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 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
282 Hyperinsulinemic hypoglycemia, familial, 4 
Autosomal recessive
Metabolic disorders HADH hydroxyacyl-CoA dehydrogenase
Reports
Updated as of Sep 08, 2025
PubMed
283 Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome 
Autosomal recessive
Metabolic disorders SLC25A15 solute carrier family 25 member 15
Reports
Updated as of Jul 20, 2024
PubMed
284 Hyperphenylalaninemia due to tetrahydrobiopterin deficiency 
Autosomal recessive
Metabolic disorders GCH1 GTP cyclohydrolase 1
Reports
Updated as of Nov 02, 2023
PubMed
285 Hyperprolinemia type 2 
Autosomal recessive
Metabolic disorders ALDH4A1 aldehyde dehydrogenase 4 family member A1
Reports
Updated as of Feb 20, 2024
PubMed
286 Hypertrophic cardiomyopathy 14 
Autosomal dominant
Cardiovascular disorders MYH6 myosin heavy chain 6
Reports
Updated as of Mar 30, 2026
PubMed
287 Hypochondroplasia 
Autosomal dominant
Bone disorders FGFR3 fibroblast growth factor receptor 3
Reports
Updated as of Jan 05, 2026
PubMed
288 Hypoparathyroidism, deafness, renal disease syndrome 
Autosomal dominant
Multisystemic disorders GATA3 GATA binding protein 3
Reports
Updated as of Mar 09, 2023
PubMed
289 Hypophosphatasia 
Autosomal dominant, Autosomal recessive
Metabolic disorders ALPL alkaline phosphatase, biomineralization associated
Reports
Updated as of Sep 05, 2023
PubMed
290 Hypotonia, infantile, with psychomotor retardation and characteristic facies 1 
Autosomal recessive
Neurodevelopmental disorders NALCN sodium leak channel, non-selective
Reports
Updated as of Feb 26, 2025
PubMed