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Anti-calsequestrin 1 Antibody

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产品名称: Anti-calsequestrin 1 Antibody
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Anti-calsequestrin 1 Antibody


Anti-calsequestrin 1 Antibody  的详细介绍
Name: Anti-calsequestrin 1 Antibody
See all calsequestrin 1 primary antibodies
Description: Rabbit polyclonal antibody to calsequestrin 1
Applications: WB, IHC
Reactivity: Human
Immunogen: Recombinant protein fragment contain a sequence corresponding to a region within amino acids 42 and 271 of Human CASQ1
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: Purified by antigen-affinity chromatography.
Formulation: Supplied in 0.1M Tris-buffered saline with 10% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.
Storage: Store at -20?C for long term preservation (recommended). Store at 4?C for short term use.
Function: Calsequestrin is a high-capacity, moderate affinity, calcium-binding protein and thus acts as an internal calcium store in muscle (PubMed:28895244). Calcium ions are bound by clusters of acidic residues at the protein surface, often at the interface between subunits. Can bind around 80 Ca(2+) ions (PubMed:28895244). Regulates the release of lumenal Ca(2+) via the calcium release channel RYR1; this plays an important role in triggering muscle contraction. Negatively regulates store-operated Ca(2+) entry (SOCE) activity (PubMed:27185316).
Tissue Specificity: Expressed in myoblasts (at protein level).
Involvement in Disease: Myopathy, vacuolar, with CASQ1 aggregates: An autosomal dominant mild muscle disorder characterized by ***** onset of muscle cramping and weakness as well as increased levels of serum creatine kinase. The disorder is not progressive, and some patients may be asymptomatic.

Myopathy, tubular aggregate, 1: A rare congenital myopathy characterized by regular arrays of membrane tubules on muscle biopsies without additional histopathological hallmarks. Tubular aggregates in muscle are structures of variable appearance consisting of an outer tubule containing either one or more microtubule-like structures or amorphous material. They may occur in a variety of circumstances, including inherited myopathies, alcohol- and drug-induced myopathies, exercise-induced cramps or muscle weakness.
Sequence Similarities: Belongs to the calsequestrin family.
Post-Translational Modification: N-glycosylated.
Cellular Location: Endoplasmic reticulum. Sarcoplasmic reticulum. Sarcoplasmic reticulum lumen. Sarcoplasmic reticulum membrane. Mitochondrion matrix.

This isoform of calsequestrin occurs in the sarcoplasmic reticulum's terminal cisternae luminal spaces of fast skeletal muscle cells. Preferentially forms linear and round aggregates in the endoplasmic reticulum (ER) of resting cells (PubMed:28895244). In a minority of cells, homogeneously detected in the ER lumen (PubMed:28895244). Colocalizes with STIM1 at endoplasmic reticulum in response to a depletion of intracellular calcium (PubMed:27185316).
Database Links:
  • Entrez Gene: 844?Human
  • Omim: 114250?Human
  • SwissProt: P31415?Human
  • Unigene: 632476?Human
    		•
    Synonyms:
  • Calmitin Antibody
  • Calmitine Antibody
  • Calsequestrin Antibody
  • Calsequestrin 1 Antibody
  • Calsequestrin 1 (fast twitch, skeletal muscle) Antibody
  • Calsequestrin, cell Antibody
  • Calsequestrin, skeletal muscle isoform Antibody
  • Calsequestrin-1 Antibody
  • CASQ Antibody
  • Casq1 Antibody
  • CASQ1_HUMAN Antibody
  • PDIB1 Antibody
  • skeletal muscle isoform Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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