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Titolo:
Malignant hyperthermia and excitation-contraction coupling
Autore:
Melzer, W; Dietze, B;
Indirizzi:
Univ Ulm, Dept Appl Physiol, D-89069 Ulm, Germany Univ Ulm Ulm Germany D-89069 lm, Dept Appl Physiol, D-89069 Ulm, Germany
Titolo Testata:
ACTA PHYSIOLOGICA SCANDINAVICA
fascicolo: 3, volume: 171, anno: 2001,
pagine: 367 - 378
SICI:
0001-6772(200103)171:3<367:MHAEC>2.0.ZU;2-W
Fonte:
ISI
Lingua:
ENG
Soggetto:
SKELETAL-MUSCLE FIBERS; CA2+ RELEASE CHANNELS; CENTRAL CORE DISEASE; SLOW CALCIUM CURRENT; INTRAMEMBRANOUS CHARGE MOVEMENT; RYANODINE RECEPTOR MUTATIONS; DEPENDENT ION CHANNELS; II-III-LOOP; DIHYDROPYRIDINE RECEPTOR; SARCOPLASMIC-RETICULUM;
Keywords:
calcium channel; calcium release; dihydropyridine receptor; excitation-contraction coupling; malignant hyperthermia; muscle disease; ryanodine receptor; skeletal muscle;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
120
Recensione:
Indirizzi per estratti:
Indirizzo: Melzer, W Univ Ulm, Dept Appl Physiol, Albert Einstein Allee 11, D-89069 Ulm, Germany Univ Ulm Albert Einstein Allee 11 Ulm Germany D-89069 , Germany
Citazione:
W. Melzer e B. Dietze, "Malignant hyperthermia and excitation-contraction coupling", ACT PHYSL S, 171(3), 2001, pp. 367-378

Abstract

Malignant hyperthermia (MH) is a state of elevated skeletal muscle metabolism that may occur during general anaesthesia in genetically pre-disposed individuals. Malignant hyperthermia results from altered control of sarcoplasmic reticulum (SR) Ca2+ release. Mutations have been identified in MH-susceptible (MHS) individuals in two key proteins of excitation-contraction (EC) coupling, the Ca2+ release channel of the SR, ryanodine receptor type 1 (RyR1) and the alpha1-subunit of the dihydropyridine receptor (DHPR, L-type Ca2+ channel). During EC coupling, the DHPR senses the plasma membrane depolarization and transmits the information to the ryanodine receptor (RyR). As a consequence, Ca2+ is released from the terminal cisternae of the SR. One of the human MH-mutations of RyR1 (Arg614Cys) is also found at the homologous location in the RyR of swine (Arg615Cys). This animal model permits the investigation of physiological consequences of the homozygously expressedmutant release channel. Of particular interest is the question of whether voltage-controlled release of Ca2+ is altered by MH-mutations in the absence of MH-triggering substances. This question has recently been addressed inthis laboratory by studying Ca2+ release under voltage clamp conditions inboth isolated human skeletal muscle fibres and porcine myotubes.

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Documento generato il 11/07/20 alle ore 04:44:49