An unusual ryanodine receptor 1 (RYR1) phenotype
نویسندگان
چکیده
منابع مشابه
Green tea catechins are potent sensitizers of ryanodine receptor type 1 (RyR1).
Catechins, polyphenols extracted from green tea leaves, have a broad range of biological activities although the specific molecular mechanisms responsible are not known. At the high experimental concentrations typically used polyphenols bind to membrane phospholipid and also are easily auto-oxidized to generate superoxide anion and semiquinones, and can adduct to protein thiols. We report that ...
متن کاملChloroform extract of hog barn dust modulates skeletal muscle ryanodine receptor calcium-release channel (RyR1).
Skeletal muscle weakness is a reported ailment in individuals working in commercial hog confinement facilities. To date, specific mechanisms responsible for this symptom remain undefined. The purpose of this study was to assess whether hog barn dust (HBD) contains components that are capable of binding to and modulating the activity of type 1 ryanodine receptor Ca2+-release channel (RyR1), a ke...
متن کاملA Structural Model of the Pore-Forming Region of the Skeletal Muscle Ryanodine Receptor (RyR1)
Ryanodine receptors (RyRs) are ion channels that regulate muscle contraction by releasing calcium ions from intracellular stores into the cytoplasm. Mutations in skeletal muscle RyR (RyR1) give rise to congenital diseases such as central core disease. The absence of high-resolution structures of RyR1 has limited our understanding of channel function and disease mechanisms at the molecular level...
متن کاملCharacterization of RyR1-slow, a ryanodine receptor specific to slow-twitch skeletal muscle.
Two distinct skeletal muscle ryanodine receptors (RyR1s) are expressed in a fiber type-specific manner in fish skeletal muscle (11). In this study, we compare [(3)H]ryanodine binding and single channel activity of RyR1-slow from fish slow-twitch skeletal muscle with RyR1-fast and RyR3 isolated from fast-twitch skeletal muscle. Scatchard plots indicate that RyR1-slow has a lower affinity for [(3...
متن کاملRyanodine Receptor
1.1 Size and Evolution The ryanodine receptor (RyR) is a selective Ca release channel that is localized to the endoplasmic reticulum (ER) in both excitable and non-excitable cells. The RyR owes its name to the plant alkaloid and high affinity ligand ryanodine. The functional RyR exists as a tetramer composed of a single unit of approximately 565kDa. To date, it is the largest known ion channel....
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ژورنال
عنوان ژورنال: Neurology
سال: 2019
ISSN: 0028-3878,1526-632X
DOI: 10.1212/wnl.0000000000007246