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
61 Cowden Syndrome 
Autosomal dominant
Cancer disorders PTEN phosphatase and tensin homolog
AKT1 AKT serine/threonine kinase 1
PIK3CA phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha
SDHD succinate dehydrogenase complex subunit D
EGFR epidermal growth factor receptor
SDHB succinate dehydrogenase complex iron sulfur subunit B
SEC23B SEC23 homolog B, COPII coat complex component
Reports
Updated as of Jul 20, 2024
PubMed
62 Cutis laxa 
Autosomal dominant, Autosomal recessive, X-linked dominant
Skin disorders ATP6V0A2 ATPase H+ transporting V0 subunit a2
LTBP4 latent transforming growth factor beta binding protein 4
EFEMP2 EGF containing fibulin extracellular matrix protein 2
FBLN5 fibulin 5
ATP7A ATPase copper transporting alpha
ELN elastin
ALDH18A1 aldehyde dehydrogenase 18 family member A1
PYCR1 pyrroline-5-carboxylate reductase 1
EFEMP1 EGF containing fibulin extracellular matrix protein 1
LTBP1 latent transforming growth factor beta binding protein 1
GBE1 1,4-alpha-glucan branching enzyme 1
Reports
Updated as of Sep 15, 2022
PubMed
63 Cystinuria 
Autosomal dominant, Autosomal recessive
Metabolic disorders SLC7A9 solute carrier family 7 member 9
SLC3A1 solute carrier family 3 member 1
CENPF centromere protein F
Reports
Updated as of Nov 30, 2023
PubMed
64 Dejerine-Sottas disease 
Autosomal dominant, Autosomal recessive
Neuromuscular disorders PRX periaxin
MPZ myelin protein zero
PMP22 peripheral myelin protein 22
EGR2 early growth response 2
Reports
Updated as of Feb 01, 2025
PubMed
65 Desmin-related myofibrillar myopathy 
Autosomal dominant, Autosomal recessive
Neuromuscular disorders DES desmin
TTN titin
Reports
Updated as of Feb 01, 2025
PubMed
66 Diamond-Blackfan anemia 
Autosomal dominant
Blood disorders RPL11 ribosomal protein L11
RPS26 ribosomal protein S26
RPS7 ribosomal protein S7
RPL5 ribosomal protein L5
RPS10 ribosomal protein S10
RPS19 ribosomal protein S19
RPS24 ribosomal protein S24
RPS17 ribosomal protein S17
RPL35A ribosomal protein L35a
RPL26 ribosomal protein L26
RPL15 ribosomal protein L15
RPS29 ribosomal protein S29
TSR2 TSR2 ribosome maturation factor
RPS28 ribosomal protein S28
RPS27 ribosomal protein S27
RPL27 ribosomal protein L27
EPO erythropoietin
TP53 tumor protein p53
RPS15A ribosomal protein S15a
RPL18 ribosomal protein L18
RPL35 ribosomal protein L35
GATA1 GATA binding protein 1
RPS20 ribosomal protein S20
HEATR3 HEAT repeat containing 3
Reports
Updated as of Nov 30, 2023
PubMed
67 Dowling-Degos disease 
Autosomal dominant
Skin disorders KRT5 keratin 5
POFUT1 protein O-fucosyltransferase 1
POGLUT1 protein O-glucosyltransferase 1
Reports
Updated as of Dec 29, 2023
PubMed
68 Duane retraction syndrome 
Autosomal dominant, Autosomal recessive
Eye disorders CHN1 chimerin 1
MAFB MAF bZIP transcription factor B
Reports
Updated as of Dec 29, 2023
PubMed
69 Dyskeratosis congenita 
Autosomal dominant, Autosomal recessive, X-linked dominant
Skin disorders NOP10 NOP10 ribonucleoprotein
NHP2 NHP2 ribonucleoprotein
TINF2 TERF1 interacting nuclear factor 2
TERC telomerase RNA component
DKC1 dyskerin pseudouridine synthase 1
TERT telomerase reverse transcriptase
WRAP53 WD repeat containing antisense to TP53
CTC1 CST telomere replication complex component 1
RTEL1 regulator of telomere elongation helicase 1
PARN poly(A)-specific ribonuclease
ACD ACD shelterin complex subunit and telomerase recruitment factor
INPP4A inositol polyphosphate-4-phosphatase type I A
TYMS thymidylate synthetase
DCLRE1B DNA cross-link repair 1B
Reports
Updated as of Dec 29, 2023
PubMed
70 Early infantile epileptic encephalopathy with suppression bursts 
Autosomal dominant, Autosomal recessive, X-linked recessive
Neurodevelopmental disorders KCNQ2 potassium voltage-gated channel subfamily Q member 2
KCNH5 potassium voltage-gated channel subfamily H member 5
SCN1A sodium voltage-gated channel alpha subunit 1
SCN8A sodium voltage-gated channel alpha subunit 8
STXBP1 syntaxin binding protein 1
SPTAN1 spectrin alpha, non-erythrocytic 1
SLC25A22 solute carrier family 25 member 22
CACNA2D2 calcium voltage-gated channel auxiliary subunit alpha2delta 2
GNAO1 G protein subunit alpha o1
PIGP phosphatidylinositol glycan anchor biosynthesis class P
ARHGEF15 Rho guanine nucleotide exchange factor 15
HCN1 hyperpolarization activated cyclic nucleotide gated potassium channel 1
ST3GAL3 ST3 beta-galactoside alpha-2,3-sialyltransferase 3
CDKL5 cyclin dependent kinase like 5
GOT2 glutamic-oxaloacetic transaminase 2
KCNB1 potassium voltage-gated channel subfamily B member 1
SNAP25 synaptosome associated protein 25
SCN2A sodium voltage-gated channel alpha subunit 2
CACNA1E calcium voltage-gated channel subunit alpha1 E
KCNC2 potassium voltage-gated channel subfamily C member 2
BSCL2 BSCL2 lipid droplet biogenesis associated, seipin
ANO4 anoctamin 4
UBA5 ubiquitin like modifier activating enzyme 5
GRIA3 glutamate ionotropic receptor AMPA type subunit 3
Reports
Updated as of Feb 26, 2025
PubMed