Structural and functional correlates of a mutation in the malignant hyperthermia-susceptible pig ryanodine receptor

James R Mickelson; C.Michael Knudson; Catharine F.H Kennedy; Ding-I Yang; Lynn A Litterer; William E Rempel; Kevin P Campbell; Charles F Louis

doi:10.1016/0014-5793(92)80208-X

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Structural and functional correlates of a mutation in the malignant hyperthermia-susceptible pig ryanodine receptor

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Structural and functional correlates of a mutation in the malignant hyperthermia-susceptible pig ryanodine receptor

James R Mickelson, C.Michael Knudson, Catharine F.H Kennedy, Ding-I Yang, Lynn A Litterer, William E Rempel, Kevin P Campbell and Charles F Louis

FEBS letters, Vol.301(1), pp.49-52

1992

DOI: 10.1016/0014-5793(92)80208-X

PMID: 1333412

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Abstract

The skeletal muscle ryanodine receptor of malignant hyperthermia-susceptible (MHS) pigs contains a mutation at residue 615 that is highly correlated with various abnormalities in the regulation of sarcoplasmic reticulum (SR) Ca 2+ channel activity. In isolated SR membranes the Arg 615 to Cys 615 ryanodine receptor mutation is now shown to be directly responsible for an altered tryptic peptide map, due to the elimination of the Arg 615 cleavage site. Furthermore, trypsin treatment released 86–99 kDa ryanodine receptor fragments encompassing residue 615 from the SR membranes. We conclude that the 86–99 kDa domain containing residue 615 is near the cytoplasmic surface of the ryanodine receptor and likely near important Ca 2+ channel regulatory sites.

Mutation

Malignant hyperthermia

Sarcoplasmic reticulum

Calcium release channel

Ryanodine receptor

Details

Title: Subtitle: Structural and functional correlates of a mutation in the malignant hyperthermia-susceptible pig ryanodine receptor
Creators: James R Mickelson - Department of Veterinary PathoBiology, University of Minnesota, St. Paul, MN 55108, USA
C.Michael Knudson - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USA
Catharine F.H Kennedy - Department of Veterinary PathoBiology, University of Minnesota, St. Paul, MN 55108, USA
Ding-I Yang - Department of Biochemistry, University of Minnesota, St. Paul, MN 55108, USA
Lynn A Litterer
William E Rempel - Department of Animal Science, University of Minnesota, St. Paul, MN 55108, USA
Kevin P Campbell - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USA
Charles F Louis - Department of Veterinary PathoBiology, University of Minnesota, St. Paul, MN 55108, USA
Resource Type: Journal article
Publication Details: FEBS letters, Vol.301(1), pp.49-52
DOI: 10.1016/0014-5793(92)80208-X
PMID: 1333412
NLM abbreviation: FEBS Lett
ISSN: 0014-5793
eISSN: 1873-3468
Publisher: Elsevier B.V
Language: English
Date published: 1992
Academic Unit: Neurology; Molecular Physiology and Biophysics; Pathology; Iowa Neuroscience Institute; Radiation Oncology
Record Identifier: 9984046822902771

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