منابع مشابه
I-13 Muscle ryanodine receptor in congenital myopathies and channelopathies
potentials and twitch force were measured in muscle strips obtained from patients and controls. Of the 36 patients, 25 presented with chronic muscle weakness of varying degrees, up to wheelchair-dependence. The weakness was associated with intracellular Na overload and edema. Older patients revealed a vacuolar myopathy or a progressive muscular dystrophy. Weakness, intracellular Na overload and...
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....
متن کاملRyanodine receptor type 3: why another ryanodine receptor isoform?
The family of ryanodine receptor (RyR) genes encodes three highly related Ca2+ release channels: RyR1, RyR2 and RyR3. Until about 10 years ago, RyRs were essentially known only for being the Ca2+ release channels of the sarcoplasmic reticulum of striated muscles, because of the high levels of expression of the RyR1 and RyR2 isoforms in skeletal and cardiac muscles, respectively. In contrast wit...
متن کاملRyanodine Receptor Adaptation
signal activates specialized Ca 2 1 release channels, the ryanodine receptors (RyRs), in the sarcoplasmic reticulum (SR). This process is called Ca 2 1 -induced Ca 2 1 release (CICR). Intuitively, the CICR process should be self-regenerating because the Ca 2 1 released from the SR should feedback and activate further SR Ca 2 1 release. However, the CICR process is precisely controlled in the he...
متن کاملPresenilin-ryanodine receptor connection.
The Presenilins (PS) gene family is composed of two members: PS1 and PS2. Mutations in PSs are linked to familial Alzheimer’s disease (FAD) (1). PSs are the catalytic subunits of the γ-secretase multimolecular complex, which mediates the intramembranous cleavage of many type I membrane proteins, including amyloid precursor protein (APP) and Notch (2, 3). Cleavage of substrates produces secreted...
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ژورنال
عنوان ژورنال: Pflügers Archiv - European Journal of Physiology
سال: 2010
ISSN: 0031-6768,1432-2013
DOI: 10.1007/s00424-010-0794-4