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Calsequestrin is a calcium-binding protein that acts as a calcium buffer within the sarcoplasmic reticulum. The protein helps hold calcium in the cisterna of the sarcoplasmic reticulum after a muscle contraction, even though the concentration of calcium in the sarcoplasmic reticulum is much higher than in the cytosol. It also helps the sarcoplasmic reticulum store an extraordinarily high amount of calcium ions. Each molecule of calsequestrin can bind 18 to 50 Ca2+ ions. Sequence analysis has suggested that calcium is not bound in distinct pockets via EF-hand motifs, but rather via presentation of a charged protein surface. Two forms of calsequestrin have been identified. The cardiac form Calsequestrin-2 (CASQ2) is present in cardiac and slow skeletal muscle and the fast skeletal form Calse

AttributesValues
rdf:type
rdfs:label
  • Calsequestrin (en)
  • Calsequestrin (de)
  • Calséquestrine (fr)
rdfs:comment
  • Calsequestrin ist ein Calcium-bindendes Protein in Muskelzellen von Wirbeltieren. Es findet sich dort in den terminalen Cisternen des sarkoplasmatischen Reticulums (SR). Pro Molekül kann es 65 Ca2+-Ionen mit geringer Affinität binden. Die Speicherkapazität sorgt dafür, dass Ca2+ im Muskel in hoher Konzentration vorliegt, aber wegen der schwachen Bindung auch schnell wieder freigesetzt werden kann. Durch die Bindung an Calsequestrin werden die Ionen aus dem Gleichgewicht entfernt. Die Ca2+-Pumpen des SR müssen somit gegen einen geringeren Konzentrationsgradienten arbeiten, die tausendfach höhere Calciumionenkonzentration in den Cisterne wird so überhaupt erst ermöglicht. (de)
  • Calsequestrin is a calcium-binding protein that acts as a calcium buffer within the sarcoplasmic reticulum. The protein helps hold calcium in the cisterna of the sarcoplasmic reticulum after a muscle contraction, even though the concentration of calcium in the sarcoplasmic reticulum is much higher than in the cytosol. It also helps the sarcoplasmic reticulum store an extraordinarily high amount of calcium ions. Each molecule of calsequestrin can bind 18 to 50 Ca2+ ions. Sequence analysis has suggested that calcium is not bound in distinct pockets via EF-hand motifs, but rather via presentation of a charged protein surface. Two forms of calsequestrin have been identified. The cardiac form Calsequestrin-2 (CASQ2) is present in cardiac and slow skeletal muscle and the fast skeletal form Calse (en)
  • La calséquestrine est une protéine à main EF qui stocke le calcium. Le complexe peut fixer jusqu'à 50 ions Ca2+. La protéine est localisée dans le réticulum endoplasmique des cellules, en particulier dans le réticulum sarcoplasmique des cellules musculaires et du myocarde. Sa capacité à complexer les ions Ca2+ permet d'augmenter considérablement la concentration calcique dans le réticulum sarcoplasmique. Cette concentration élevée est normalement cytotoxique. Elle temporise les échanges de calcium avec le cytosol. (fr)
foaf:name
  • calsequestrin 1 (fast-twitch, skeletal muscle) (en)
  • calsequestrin 2 (cardiac muscle) (en)
name
  • Calsequestrin (en)
  • calsequestrin 1 (en)
  • calsequestrin 2 (en)
foaf:depiction
  • http://commons.wikimedia.org/wiki/Special:FilePath/Calsequestrin1.png
  • http://commons.wikimedia.org/wiki/Special:FilePath/PDB_1a8y_EBI.jpg
dcterms:subject
Wikipage page ID
Wikipage revision ID
Link from a Wikipage to another Wikipage
Link from a Wikipage to an external page
sameAs
dbp:wikiPageUsesTemplate
thumbnail
OMIM
PROSITE
  • PDOC00675 (en)
SCOP
AltSymbols
  • CASQ (en)
caption
  • Calsequestrin monomer showing the three repeating calsequestrin domains (en)
  • crystal structure of calsequestrin from rabbit skeletal muscle sarcoplasmic reticulum at 2.4 a resolution (en)
InterPro
  • IPR001393 (en)
Pfam
  • PF01216 (en)
Pfam clan
  • CL0172 (en)
symbol
  • CASQ1 (en)
  • CASQ2 (en)
  • Calsequestrin (en)
UniProt
  • O14958 (en)
  • P31415 (en)
has abstract
  • Calsequestrin ist ein Calcium-bindendes Protein in Muskelzellen von Wirbeltieren. Es findet sich dort in den terminalen Cisternen des sarkoplasmatischen Reticulums (SR). Pro Molekül kann es 65 Ca2+-Ionen mit geringer Affinität binden. Die Speicherkapazität sorgt dafür, dass Ca2+ im Muskel in hoher Konzentration vorliegt, aber wegen der schwachen Bindung auch schnell wieder freigesetzt werden kann. Durch die Bindung an Calsequestrin werden die Ionen aus dem Gleichgewicht entfernt. Die Ca2+-Pumpen des SR müssen somit gegen einen geringeren Konzentrationsgradienten arbeiten, die tausendfach höhere Calciumionenkonzentration in den Cisterne wird so überhaupt erst ermöglicht. Die im Herzmuskel vorkommende, leicht veränderte Isoform des Calsequestrin-1 wird Calsequestrin-2 genannt. Mutationen in dessen Gen (CASQ2) können erbliche ventrikuläre Tachykardie verursachen. (de)
  • Calsequestrin is a calcium-binding protein that acts as a calcium buffer within the sarcoplasmic reticulum. The protein helps hold calcium in the cisterna of the sarcoplasmic reticulum after a muscle contraction, even though the concentration of calcium in the sarcoplasmic reticulum is much higher than in the cytosol. It also helps the sarcoplasmic reticulum store an extraordinarily high amount of calcium ions. Each molecule of calsequestrin can bind 18 to 50 Ca2+ ions. Sequence analysis has suggested that calcium is not bound in distinct pockets via EF-hand motifs, but rather via presentation of a charged protein surface. Two forms of calsequestrin have been identified. The cardiac form Calsequestrin-2 (CASQ2) is present in cardiac and slow skeletal muscle and the fast skeletal form Calsequestrin-1(CASQ1) is found in fast skeletal muscle. The release of calsequestrin-bound calcium (through a calcium release channel) triggers muscle contraction. The active protein is not highly structured, more than 50% of it adopting a random coil conformation. When calcium binds there is a structural change whereby the alpha-helical content of the protein increases from 3 to 11%. Both forms of calsequestrin are phosphorylated by casein kinase 2, but the cardiac form is phosphorylated more rapidly and to a higher degree. Calsequestrin is also secreted in the gut where it deprives bacteria of calcium ions.. (en)
  • La calséquestrine est une protéine à main EF qui stocke le calcium. Le complexe peut fixer jusqu'à 50 ions Ca2+. La protéine est localisée dans le réticulum endoplasmique des cellules, en particulier dans le réticulum sarcoplasmique des cellules musculaires et du myocarde. Sa capacité à complexer les ions Ca2+ permet d'augmenter considérablement la concentration calcique dans le réticulum sarcoplasmique. Cette concentration élevée est normalement cytotoxique. Elle temporise les échanges de calcium avec le cytosol. Une maladie rare, la « myopathie due à une surcharge en calséquestrine et SERCA1 », a été exceptionnellement décrite. Ce syndrome réalise une myopathie qui serait due à un excès de calséquestrine et de SERCA1. (fr)
Arm
  • p (en)
  • q (en)
band
Chromosome
EntrezGene
HGNCid
LocusSupplementaryData
  • -p11 (en)
PDB
RefSeq
  • NM_001231 (en)
  • NM_001232 (en)
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