CHRND Chromosome 2

Cholinergic receptor nicotinic delta subunit
84 variants 84 Health Risk

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What This Gene Does
The acetylcholine receptor of muscle has 5 subunits of 4 different types: 2 alpha and 1 each of beta, gamma and delta subunits. After acetylcholine binding, the receptor undergoes an extensive conformation change that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane. Defects in this gene are a cause of multiple pterygium syndrome lethal type (MUPSL), congenital myasthenic syndrome slow-channel type (SCCMS), and congenital myasthenic syndrome fast-channel type (FCCMS). Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2015]
Gene Info
Gene Group
Cholinergic receptors nicotinic subunits
Locus Type
gene with protein product
Location
2q37.1
Ensembl
ENSG00000135902
Associated Conditions (15)
Congenital myasthenic syndrome
Lethal multiple pterygium syndrome
Inborn genetic diseases
Congenital myasthenic syndrome 3A
Congenital myasthenic syndrome 3B
Congenital myasthenic syndrome 3C
CHRND-related disorder
Slow-Channel Congenital Myasthenia Syndrome
Centronuclear myopathy
Ptosis
Breathing dysregulation
Muscle weakness
Dyspnea
Arthrogryposis multiplex congenita
Fetal akinesia deformation sequence 1
Key Variants
RS114463490
Conflicting classifications of pathogenicity
Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
Health Risk
RS1352698710
Conflicting classifications of pathogenicity
Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
Health Risk
RS138609765
Conflicting classifications of pathogenicity
Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
Health Risk
RS139190636
Conflicting classifications of pathogenicity
Lethal multiple pterygium syndrome, Inborn genetic diseases, Lethal multiple pterygium syndrome
Health Risk
RS139722128
Conflicting classifications of pathogenicity
Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
Health Risk
RS142063328
Conflicting classifications of pathogenicity
Congenital myasthenic syndrome, Congenital myasthenic syndrome 3A, Congenital myasthenic syndrome 3B
Health Risk
RS142531974
Conflicting classifications of pathogenicity
Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
Health Risk
RS143246852
Conflicting classifications of pathogenicity
Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
Health Risk
RS144310284
Conflicting classifications of pathogenicity
Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
Health Risk
RS144433265
Conflicting classifications of pathogenicity
Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
Health Risk
RS145955590
Conflicting classifications of pathogenicity
Congenital myasthenic syndrome 3C, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome 3B
Health Risk
RS146205427
Conflicting classifications of pathogenicity
Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
Health Risk
All Variants (84)
RSID Category Clinical Significance Conditions
RS114463490 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS1352698710 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS138609765 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
RS139190636 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Inborn genetic diseases, Lethal multiple pterygium syndrome
RS139722128 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS142063328 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Congenital myasthenic syndrome 3A, Congenital myasthenic syndrome 3B
RS142531974 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS143246852 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS144310284 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS144433265 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
RS145955590 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome 3C, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome 3B
RS146205427 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS146416320 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Inborn genetic diseases, Lethal multiple pterygium syndrome
RS147452877 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Inborn genetic diseases, Lethal multiple pterygium syndrome
RS148869069 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS148939701 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, CHRND-related disorder
RS150208750 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
RS1553574327 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS1553575435 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS1574633904 Health Risk Conflicting classifications of pathogenicity Slow-Channel Congenital Myasthenia Syndrome, Lethal multiple pterygium syndrome, Centronuclear myopathy
RS199508773 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS199538903 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS199592458 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS200986300 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
RS201733876 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome 3A
RS202209156 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, CHRND-related disorder, Lethal multiple pterygium syndrome
RS2229194 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Congenital myasthenic syndrome 3A
RS2853458 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS367837942 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS368695808 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS369026633 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS369869476 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Inborn genetic diseases, Lethal multiple pterygium syndrome
RS373187583 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome 3B, Congenital myasthenic syndrome 3C
RS373578965 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, CHRND-related disorder
RS373581541 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS376642208 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS545298641 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
RS547787086 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
RS554645326 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS56203086 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS564930869 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Inborn genetic diseases, Lethal multiple pterygium syndrome
RS567130034 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS749757968 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS749866545 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
RS754087173 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Lethal multiple pterygium syndrome
RS756537437 Health Risk Conflicting classifications of pathogenicity Lethal multiple pterygium syndrome, Congenital myasthenic syndrome, Lethal multiple pterygium syndrome
RS776972228 Health Risk Conflicting classifications of pathogenicity Congenital myasthenic syndrome, Lethal multiple pterygium syndrome, Congenital myasthenic syndrome
RS797045474 Health Risk Conflicting classifications of pathogenicity
RS1057518957 Health Risk Likely pathogenic Ptosis, Breathing dysregulation, Muscle weakness
RS1057518958 Health Risk Likely pathogenic Ptosis, Breathing dysregulation, Muscle weakness
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