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
191 Pachyonychia congenita 
Autosomal dominant
Skin disorders KRT17 keratin 17
KRT16 keratin 16
KRT6B keratin 6B
KRT6A keratin 6A
Reports
Updated as of Jan 04, 2024
PubMed
192 Papillary renal cell carcinoma 
Autosomal dominant
Cancer disorders VHL von Hippel-Lindau tumor suppressor
MET MET proto-oncogene, receptor tyrosine kinase
SDHB succinate dehydrogenase complex iron sulfur subunit B
PTEN phosphatase and tensin homolog
ERBB2 erb-b2 receptor tyrosine kinase 2
ARAF A-Raf proto-oncogene, serine/threonine kinase
BRAF B-Raf proto-oncogene, serine/threonine kinase
PIK3CA phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha
RHEB Ras homolog, mTORC1 binding
ELOC elongin C
TP53 tumor protein p53
MTOR mechanistic target of rapamycin kinase
Reports
Updated as of Jan 04, 2024
PubMed
193 Paroxysmal nocturnal hemoglobinuria 
Blood disorders PIGA phosphatidylinositol glycan anchor biosynthesis class A
LOC110806306 telomerase RNA component (TERC) promoter
Reports
Updated as of Feb 20, 2024
PubMed
194 Pemphigus vulgaris 
Autosomal dominant
Skin disorders HLA-DRB1 major histocompatibility complex, class II, DR beta 1
HLA-DQB1 major histocompatibility complex, class II, DQ beta 1
HLA-B major histocompatibility complex, class I, B
DSG3 desmoglein 3
ST18 ST18 C2H2C-type zinc finger transcription factor
TNF tumor necrosis factor
Reports
Updated as of Sep 05, 2023
PubMed
195 Peroxisome biogenesis disorder 
Autosomal recessive
Neurodegenerative disorders PEX26 peroxisomal biogenesis factor 26
PEX16 peroxisomal biogenesis factor 16
PEX3 peroxisomal biogenesis factor 3
PEX10 peroxisomal biogenesis factor 10
PEX1 peroxisomal biogenesis factor 1
PEX14 peroxisomal biogenesis factor 14
PEX13 peroxisomal biogenesis factor 13
PEX12 peroxisomal biogenesis factor 12
PEX7 peroxisomal biogenesis factor 7
PEX6 peroxisomal biogenesis factor 6
PEX5 peroxisomal biogenesis factor 5
PEX2 peroxisomal biogenesis factor 2
PEX19 peroxisomal biogenesis factor 19
PEX11B peroxisomal biogenesis factor 11 beta
Reports
Updated as of Sep 05, 2023
PubMed
196 Perrault syndrome 1 
Autosomal recessive
Endocrine disorders HSD17B4 hydroxysteroid 17-beta dehydrogenase 4
PRORP protein only RNase P catalytic subunit
FBN1 fibrillin 1
CLPP caseinolytic mitochondrial matrix peptidase proteolytic subunit
Reports
Updated as of Jan 04, 2024
PubMed
197 Persistent mullerian duct syndrome 
Autosomal recessive
Endocrine disorders AMH anti-Mullerian hormone
AMHR2 anti-Mullerian hormone receptor type 2
Reports
Updated as of Sep 05, 2023
PubMed
198 Pfeiffer syndrome 
Autosomal dominant
Bone disorders FGFR2 fibroblast growth factor receptor 2
FGFR1 fibroblast growth factor receptor 1
Reports
Updated as of Mar 09, 2023
PubMed
199 Phenylketonuria 
Autosomal recessive
Metabolic disorders PAH phenylalanine hydroxylase
COL1A1 collagen type I alpha 1 chain
Reports
Updated as of Sep 15, 2022
PubMed
200 Phytanic acid storage disease 
Autosomal recessive
Metabolic disorders PHYH phytanoyl-CoA 2-hydroxylase
PEX7 peroxisomal biogenesis factor 7
Reports
Updated as of Feb 20, 2024
PubMed