T lymphocytes from malignant hyperthermia-susceptible mice display aberrations in intracellular calcium signaling and mitochondrial function.

ABSTRACT: Gain-of-function RyR1-p.R163C mutation in ryanodine receptors type 1 (RyR1) deregulates Ca²⁺ signaling and mitochondrial function in skeletal muscle and causes malignant hyperthermia in humans and mice under triggering conditions. We investigated whether T lymphocytes from heterozygous RyR1-p.R163C knock-in mutant mice (HET T cells) display measurable aberrations in resting cytosolic Ca²⁺ concentration ([Ca²⁺]_i), Ca²⁺ release from the store, store-operated Ca²⁺ entry (SOCE), and mitochondrial inner membrane potential (ΔΨ_m) compared with T lymphocytes from wild-type mice (WT T cells). We explored whether these variables can be used to distinguish between T cells with normal and altered RyR1 genotype. HET and WT T c

SUBMITTER: Yang L 

PROVIDER: S-EPMC7840221 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature