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
251 Sudden infant death syndrome 
Autosomal recessive
Metabolic disorders KCNQ1 potassium voltage-gated channel subfamily Q member 1
KCNH2 potassium voltage-gated channel subfamily H member 2
SCN5A sodium voltage-gated channel alpha subunit 5
SCN4B sodium voltage-gated channel beta subunit 4
SCN1A sodium voltage-gated channel alpha subunit 1
DEPDC5 DEP domain containing 5, GATOR1 subcomplex subunit
CALM2 calmodulin 2
Reports
Updated as of Mar 09, 2023
PubMed
252 Sulfite oxidase deficiency 
Autosomal recessive
Metabolic disorders SUOX sulfite oxidase
GPHN gephyrin
MOCS2 molybdenum cofactor synthesis 2
MOCS1 molybdenum cofactor synthesis 1
Reports
Updated as of Feb 20, 2024
PubMed
253 Systemic lupus erythematosus 
Autosomal dominant
Immune disorders IRF5 interferon regulatory factor 5
BLK BLK proto-oncogene, Src family tyrosine kinase
TREX1 three prime repair exonuclease 1
SOCS1 suppressor of cytokine signaling 1
TLR7 toll like receptor 7
Reports
Updated as of May 24, 2023
PubMed
254 Systemic-Onset Juvenile Idiopathic Arthritis 
Bone disorders S100A9 S100 calcium binding protein A9
IL6 interleukin 6
HLA-DRB1 major histocompatibility complex, class II, DR beta 1
LACC1 laccase domain containing 1
Reports
Updated as of Sep 05, 2023
PubMed
255 Takayasu arteritis 
Cardiovascular disorders MLXIPL MLX interacting protein like
IL12B interleukin 12B
HLA-B major histocompatibility complex, class I, B
Reports
Updated as of Dec 29, 2023
PubMed
256 Tangier disease 
Autosomal recessive
Metabolic disorders ABCA1 ATP binding cassette subfamily A member 1
APOA1 apolipoprotein A1
Reports
Updated as of Dec 29, 2023
PubMed
257 Tay-Sachs disease 
Autosomal recessive
Metabolic disorders/Lysosomal storage disorders GM2A ganglioside GM2 activator
HEXA hexosaminidase subunit alpha
Reports
Updated as of Sep 15, 2022
PubMed
258 Testicular agenesis 
Endocrine disorders SRY sex determining region Y
NR5A1 nuclear receptor subfamily 5 group A member 1
INSL3 insulin like 3
RXFP2 relaxin family peptide receptor 2
Reports
Updated as of Mar 09, 2023
PubMed
259 Tetralogy of Fallot 
Autosomal dominant
Cardiovascular disorders JAG1 jagged canonical Notch ligand 1
NKX2-5 NK2 homeobox 5
GATA4 GATA binding protein 4
GATA6 GATA binding protein 6
ZFPM2 zinc finger protein, FOG family member 2
EPHB4 EPH receptor B4
NOTCH1 notch receptor 1
TPM1 tropomyosin 1
TBX1 T-box transcription factor 1
NIPBL NIPBL cohesin loading factor
MT-CO1 mitochondrially encoded cytochrome c oxidase I
MT-CO2 mitochondrially encoded cytochrome c oxidase II
MT-CO3 mitochondrially encoded cytochrome c oxidase III
ROBO1 roundabout guidance receptor 1
FLNC filamin C
Reports
Updated as of Dec 29, 2023
PubMed
260 Thrombophilia due to thrombin defect 
Autosomal dominant
Blood disorders F2 coagulation factor II, thrombin
F5 coagulation factor V
Reports
Updated as of Feb 26, 2025
PubMed