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
121 Hirschsprung disease 
Autosomal dominant, Autosomal recessive
Gastrointestinal disorders PHOX2B paired like homeobox 2B
ECE1 endothelin converting enzyme 1
RET ret proto-oncogene
EDNRB endothelin receptor type B
EDN3 endothelin 3
SOX10 SRY-box transcription factor 10
FBN1 fibrillin 1
IHH Indian hedgehog signaling molecule
TBATA thymus, brain and testes associated
NCLN nicalin
NUP98 nucleoporin 98 and 96 precursor
DENND3 DENN domain containing 3
GDNF glial cell derived neurotrophic factor
Reports
Updated as of Mar 09, 2023
PubMed
122 Histiocytic medullary reticulosis 
Autosomal recessive
Cancer disorders DCLRE1C DNA cross-link repair 1C
RAG2 recombination activating 2
RAG1 recombination activating 1
IL7R interleukin 7 receptor
Reports
Updated as of Sep 08, 2025
PubMed
123 Hurler syndrome 
Autosomal recessive
Metabolic disorders/Lysosomal storage disorders IDUA alpha-L-iduronidase
PITX1 paired like homeodomain 1
Reports
Updated as of May 24, 2023
PubMed
124 Hyper-IgE syndrome 
Autosomal dominant, Autosomal recessive
Immune disorders STAT3 signal transducer and activator of transcription 3
PGM3 phosphoglucomutase 3
DOCK8 dedicator of cytokinesis 8
STAT6 signal transducer and activator of transcription 6
Reports
Updated as of Feb 26, 2025
PubMed
125 Hyperkalemic periodic paralysis 
Autosomal dominant
Metabolic disorders SCN4A sodium voltage-gated channel alpha subunit 4
CLCN1 chloride voltage-gated channel 1
Reports
Updated as of Feb 01, 2025
PubMed
126 Hypertrophic osteoarthropathy 
Autosomal recessive
Bone disorders HPGD 15-hydroxyprostaglandin dehydrogenase
SLCO2A1 solute carrier organic anion transporter family member 2A1
Reports
Updated as of Sep 05, 2023
PubMed
127 Hypokalemic periodic paralysis, type 1 
Autosomal dominant
Metabolic disorders CACNA1S calcium voltage-gated channel subunit alpha1 S
CLCN1 chloride voltage-gated channel 1
Reports
Updated as of Feb 20, 2024
PubMed
128 Immune thrombocytopenia 
Autosomal dominant
Blood disorders FCGR2B Fc gamma receptor IIb
FCGR2C Fc gamma receptor IIc (gene/pseudogene)
Reports
Updated as of May 24, 2023
PubMed
129 Infantile cortical hyperostosis 
Autosomal dominant
Bone disorders COL1A1 collagen type I alpha 1 chain
A4GALT alpha 1,4-galactosyltransferase (P1PK blood group)
Reports
Updated as of Nov 30, 2023
PubMed
130 Intestinal hypomagnesemia 1 
Autosomal recessive
Metabolic disorders TRPM6 transient receptor potential cation channel subfamily M member 6
TRPM7 transient receptor potential cation channel subfamily M member 7
Reports
Updated as of Jul 20, 2024
PubMed