Hypertrophy in Skeletal Myotubes Induced by Junctophilin-2 Mutant, Y141H, Involves an Increase in Store-operated Ca2+ Entry via Orai1

Junctophilins (JPs) play an important role in the formation of junctional membrane complexes (JMC) in striated muscle by physically linking the transverse-tubule and sarcoplasmic reticulum (SR) membranes. Researchers have found five JP2 mutants in humans with hypertrophic cardiomyopathy. Among these...

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Veröffentlicht in:	The Journal of biological chemistry 2012-04, Vol.287 (18), p.14336-14348
Hauptverfasser:	Woo, Jin Seok, Cho, Chung-Hyun, Lee, Keon Jin, Kim, Do Han, Ma, Jianjie, Lee, Eun Hui
Format:	Artikel
Sprache:	eng
Schlagworte:	Amino Acid Substitution Animals Calcium - metabolism Calcium Channels - genetics Calcium Channels - metabolism Calcium Imaging Calcium Signaling Cardiomyopathy, Hypertrophic - genetics Cardiomyopathy, Hypertrophic - metabolism Cardiomyopathy, Hypertrophic - pathology Excitation-Contraction Coupling Humans Hypertrophy Intracellular Membranes - metabolism Intracellular Membranes - pathology Junctional Membrane Complexes Junctophilin-2 Mutant Membrane Biology Membrane Proteins - genetics Membrane Proteins - metabolism Mice Muscle Fibers, Skeletal - metabolism Muscle Fibers, Skeletal - pathology Muscle Proteins - genetics Muscle Proteins - metabolism Mutation, Missense Myopathies, Structural, Congenital - genetics Myopathies, Structural, Congenital - metabolism Myopathies, Structural, Congenital - pathology ORAI1 Protein Ryanodine Receptor Ryanodine Receptor Calcium Release Channel - genetics Ryanodine Receptor Calcium Release Channel - metabolism Sarcoplasmic Reticulum - genetics Sarcoplasmic Reticulum - metabolism Sarcoplasmic Reticulum - pathology Skeletal Muscle Store-operated Ca2+ Entry
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