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
171 Multicystic dysplastic kidneys 
Nephrological disorders HNF1B HNF1 homeobox B
PAX2 paired box 2
Reports
Updated as of Sep 05, 2023
PubMed
172 Multiminicore myopathy 
Autosomal dominant, Autosomal recessive
Neuromuscular disorders RYR1 ryanodine receptor 1
TTN titin
Reports
Updated as of Feb 01, 2025
PubMed
173 Multiple acyl-CoA dehydrogenase deficiency 
Autosomal recessive
Metabolic disorders ETFDH electron transfer flavoprotein dehydrogenase
ETFA electron transfer flavoprotein subunit alpha
FLAD1 flavin adenine dinucleotide synthetase 1
ETFB electron transfer flavoprotein subunit beta
MADD MAP kinase activating death domain
Reports
Updated as of Mar 09, 2023
PubMed
174 Multiple endocrine neoplasia, type 1 
Autosomal dominant
Cancer disorders MEN1 menin 1
CDC73 cell division cycle 73
Reports
Updated as of Sep 05, 2023
PubMed
175 Multiple myeloma 
Somatic mutation
Cancer disorders FGFR3 fibroblast growth factor receptor 3
BRAF B-Raf proto-oncogene, serine/threonine kinase
TP53 tumor protein p53
TRAF5 TNF receptor associated factor 5
CDKN2C cyclin dependent kinase inhibitor 2C
BARD1 BRCA1 associated RING domain 1
HDAC4 histone deacetylase 4
DNMT3A DNA methyltransferase 3 alpha
TET3 tet methylcytosine dioxygenase 3
CRBN cereblon
MST1R macrophage stimulating 1 receptor
BAP1 BRCA1 associated protein 1
TET2 tet methylcytosine dioxygenase 2
FAT1 FAT atypical cadherin 1
IL7R interleukin 7 receptor
SGK1 serum/glucocorticoid regulated kinase 1
LATS1 large tumor suppressor kinase 1
H3C1 H3 clustered histone 1
H1-4 H1.4 linker histone, cluster member
H2AC16 H2A clustered histone 16
H2AC17 H2A clustered histone 17
KMT2C lysine methyltransferase 2C
RXRA retinoid X receptor alpha
YAP1 Yes1 associated transcriptional regulator
ATM ATM serine/threonine kinase
SH2B3 SH2B adaptor protein 3
NCOR2 nuclear receptor corepressor 2
FLT3 fms related receptor tyrosine kinase 3
DIS3 DIS3 homolog, exosome endoribonuclease and 3'-5' exoribonuclease
NKX2-1 NK2 homeobox 1
ARID4A AT-rich interaction domain 4A
MGA MAX dimerization protein MGA
CREBBP CREB binding protein
CYLD CYLD lysine 63 deubiquitinase
NF1 neurofibromin 1
TCF3 transcription factor 3
PIK3R2 phosphoinositide-3-kinase regulatory subunit 2
ERF ETS2 repressor factor
SAMHD1 SAM and HD domain containing deoxynucleoside triphosphate triphosphohydrolase 1
AURKA aurora kinase A
BCORL1 BCL6 corepressor like 1
EIF1AX eukaryotic translation initiation factor 1A X-linked
KDM5C lysine demethylase 5C
P2RY8 P2Y receptor family member 8
Reports
Updated as of Feb 01, 2024
PubMed
176 Multiple sulfatase deficiency 
Autosomal recessive
Metabolic disorders/Lysosomal storage disorders SUMF1 sulfatase modifying factor 1
SUMF2 sulfatase modifying factor 2
Reports
Updated as of Feb 01, 2024
PubMed
177 Myelodysplastic syndrome 
Blood disorders MT-TL1 mitochondrially encoded tRNA leucine 1 (UUA/G)
MT-CO1 mitochondrially encoded cytochrome c oxidase I
GATA2 GATA binding protein 2
NRAS NRAS proto-oncogene, GTPase
SF3B1 splicing factor 3b subunit 1
FLT3 fms related receptor tyrosine kinase 3
DNMT3A DNA methyltransferase 3 alpha
IDH1 isocitrate dehydrogenase (NADP(+)) 1
IDH2 isocitrate dehydrogenase (NADP(+)) 2
TET2 tet methylcytosine dioxygenase 2
DDX41 DEAD-box helicase 41
ASXL1 ASXL transcriptional regulator 1
SAMD9 sterile alpha motif domain containing 9
Reports
Updated as of Feb 20, 2024
PubMed
178 Myeloproliferative disorder 
Blood disorders FGFR3 fibroblast growth factor receptor 3
DNMT3A DNA methyltransferase 3 alpha
PDGFRB platelet derived growth factor receptor beta
Reports
Updated as of Feb 20, 2024
PubMed
179 Nephronophthisis 
Autosomal recessive
Nephrological disorders XPNPEP3 X-prolyl aminopeptidase 3
TMEM67 transmembrane protein 67
NEK8 NIMA related kinase 8
NPHP3 nephrocystin 3
NPHP4 nephrocystin 4
NPHP1 nephrocystin 1
WDR19 WD repeat domain 19
TTC21B tetratricopeptide repeat domain 21B
CEP164 centrosomal protein 164
ANKS6 ankyrin repeat and sterile alpha motif domain containing 6
GLIS2 GLIS family zinc finger 2
CEP83 centrosomal protein 83
DCDC2 doublecortin domain containing 2
RPGRIP1L RPGRIP1 like
IQCB1 IQ motif containing B1
INVS inversin
MAPKBP1 mitogen-activated protein kinase binding protein 1
RBM48 RNA binding motif protein 48
INCENP inner centromere protein
FAM186B family with sequence similarity 186 member B
PIAS1 protein inhibitor of activated STAT 1
CEP290 centrosomal protein 290
ZNF423 zinc finger protein 423
AHI1 Abelson helper integration site 1
RMND1 required for meiotic nuclear division 1 homolog
MKKS MKKS centrosomal shuttling protein
SLC41A1 solute carrier family 41 member 1
ADAMTS9 ADAM metallopeptidase with thrombospondin type 1 motif 9
Reports
Updated as of Nov 02, 2023
PubMed
180 Neuroblastoma 
Cancer disorders KIF1B kinesin family member 1B
PHOX2B paired like homeobox 2B
ALK ALK receptor tyrosine kinase
FGFR1 fibroblast growth factor receptor 1
MYC MYC proto-oncogene, bHLH transcription factor
BRCA2 BRCA2 DNA repair associated
SMARCA4 SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily a, member 4
PTCH1 patched 1
Reports
Updated as of Jan 04, 2024
PubMed