RYR1 Chromosome 19

Ryanodine receptor 1
1315 variants 24 Drug Response 1291 Health Risk

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What This Gene Does
This gene encodes a ryanodine receptor found in skeletal muscle. The encoded protein functions as a calcium release channel in the sarcoplasmic reticulum but also serves to connect the sarcoplasmic reticulum and transverse tubule. Mutations in this gene are associated with malignant hyperthermia susceptibility, central core disease, and minicore myopathy with external ophthalmoplegia. Alternatively spliced transcripts encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
Gene Info
Gene Group
"Ryanodine receptors|Protein phosphatase 1 regulatory subunits"
Locus Type
gene with protein product
Location
19q13.2
Ensembl
ENSG00000196218
Associated Conditions (74)
Central core myopathy
RYR1-related disorder
King Denborough syndrome
desflurane response - Toxicity
halothane response - Toxicity
enflurane response - Toxicity
isoflurane response - Toxicity
methoxyflurane response - Toxicity
sevoflurane response - Toxicity
succinylcholine response - Toxicity
RYR1-related myopathy
Malignant hyperthermia
susceptibility to
1
Congenital multicore myopathy with external ophthalmoplegia
Malignant hyperthermia of anesthesia
Congenital myopathy with fiber type disproportion
Inborn genetic diseases
Congenital myopathy
Myopathy
+54 more conditions
Key Variants
RS118192124
Likely pathogenic; drug response
Central core myopathy, RYR1-related disorder, King Denborough syndrome
Drug Response
RS121918596
Likely pathogenic; drug response
Malignant hyperthermia, susceptibility to, 1
Drug Response
RS193922748
Likely pathogenic; drug response
RYR1-related disorder, sevoflurane response - Toxicity, Malignant hyperthermia
Drug Response
RS193922762
Likely pathogenic; drug response
Malignant hyperthermia, susceptibility to, 1
Drug Response
RS193922770
Likely pathogenic; drug response
RYR1-related disorder, desflurane response - Toxicity, halothane response - Toxicity
Drug Response
RS193922818
Likely pathogenic; drug response
RYR1-related disorder, Malignant hyperthermia, susceptibility to
Drug Response
RS193922832
Likely pathogenic; drug response
RYR1-related disorder, sevoflurane response - Toxicity, desflurane response - Toxicity
Drug Response
RS193922876
Likely pathogenic; drug response
desflurane response - Toxicity, enflurane response - Toxicity, succinylcholine response - Toxicity
Drug Response
RS193922878
Likely pathogenic; drug response
desflurane response - Toxicity, halothane response - Toxicity, isoflurane response - Toxicity
Drug Response
RS28933397
Likely pathogenic; drug response
Malignant hyperthermia, susceptibility to, 1
Drug Response
RS118192122
Pathogenic; drug response
Central core myopathy, Malignant hyperthermia, susceptibility to
Drug Response
RS118192161
Pathogenic; drug response
Malignant hyperthermia, susceptibility to, 1
Drug Response
All Variants (1315)
RSID Category Clinical Significance Conditions
RS193922801 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS193922805 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS193922807 Health Risk Likely pathogenic enflurane response - Toxicity, desflurane response - Toxicity, halothane response - Toxicity
RS193922809 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS193922810 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS193922812 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS193922816 Health Risk Likely pathogenic Malignant hyperthermia of anesthesia, RYR1-related disorder, Malignant hyperthermia
RS193922826 Health Risk Likely pathogenic RYR1-related disorder, Malignant hyperthermia of anesthesia, Malignant hyperthermia
RS193922842 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS193922844 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS193922847 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS193922867 Health Risk Likely pathogenic RYR1-related disorder, Malignant hyperthermia of anesthesia, Central core myopathy
RS193922892 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS193922893 Health Risk Likely pathogenic Central core myopathy, Central core myopathy
RS1966917399 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1967335085 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1967488222 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS1967600281 Health Risk Likely pathogenic Hydrops fetalis, Hydrops fetalis
RS1967603354 Health Risk Likely pathogenic Congenital myopathy with fiber type disproportion, Congenital myopathy with fiber type disproportion
RS1967928524 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1968291835 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1968540857 Health Risk Likely pathogenic Congenital multicore myopathy with external ophthalmoplegia, Congenital multicore myopathy with external ophthalmoplegia
RS1969238474 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1969278145 Health Risk Likely pathogenic
RS1970195394 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS1970417447 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1970762402 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1971306200 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1971575594 Health Risk Likely pathogenic Central core myopathy, Central core myopathy
RS1972803770 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1973672109 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS1974148669 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder, RYR1-related disorder
RS1974497223 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS200069592 Health Risk Likely pathogenic Malignant hypothermia, RYR1-related disorder, Malignant hyperthermia
RS200576795 Health Risk Likely pathogenic Congenital multicore myopathy with external ophthalmoplegia, Congenital multicore myopathy with external ophthalmoplegia
RS2145361853 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS2145381188 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS2145447772 Health Risk Likely pathogenic Malignant hyperthermia of anesthesia, RYR1-related disorder, Malignant hyperthermia of anesthesia
RS2145482463 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS2145585588 Health Risk Likely pathogenic
RS2145586850 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS2145601896 Health Risk Likely pathogenic Malignant hyperthermia, susceptibility to, 1
RS2145614578 Health Risk Likely pathogenic
RS2145643810 Health Risk Likely pathogenic
RS2145681468 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS2145723826 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS2145774562 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS2145790333 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS2145831560 Health Risk Likely pathogenic RYR1-related disorder, RYR1-related disorder
RS2145845399 Health Risk Likely pathogenic
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