Calsequestrin-2 Protein | CASQ2 protein

Calsequestrin-2 (CASQ2, CSQ, Calsequestrin cardiac muscle isoform)

Cat. Num. AAA634874

• Gene Names: CASQ2; PDIB2
• Purity: Highly Purified; 90% by SDS-PAGE
• Synonyms: Calsequestrin-2; Calsequestrin-2 (CASQ2; CSQ; Calsequestrin cardiac muscle isoform); CASQ2 protein

• Host: Canine heart
• Purity/Purification: Highly Purified; 90% by SDS-PAGE
• Form/Format: Supplied as a lyophilized powder from 10mM Tris-HCl, 1mM EGTA. Reconstitute in sterile dH20 to 0.1mg/ml.

• Application Notes: Suitable for use in Western Blot, Absorption and Immunohistochemistry.
• Preparation and Storage: Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with sterile ddH2O. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Related Product Information for CASQ2 protein

Calsequestrin is the major calcium storage protein of the sarcoplasmic reticulum. Intraluminar Ca2+ binds to calsequestrin during diastole to prevent Ca2+ precipitation and to lower its free ionic concentration to facilitate efficient storage. During systole, Calsequestrin coordinately releases ~40-50 Ca2+ ions per molecule for each contraction-relaxation cycle by an uncertain mechanism. Calsequestrin has been shown to be of major importance in the regulation of cardiac excitation-contraction coupling.

Product Categories/Family for CASQ2 protein

Molecular Biology; MB-Binding Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 119395727
• NCBI GeneID: 845
• NCBI Accession #: NP_001223.2
• NCBI GenBank Nucleotide #: NM_001232.3
• UniProt Accession #: O14958; Q5T1D2; Q8TBW8; B2R7M6
• Molecular Weight: 46,436 Da
• NCBI Official Full Name: calsequestrin-2
• NCBI Official Synonym Full Names: calsequestrin 2 (cardiac muscle)
• NCBI Official Symbol: CASQ2
• NCBI Official Synonym Symbols: PDIB2
• NCBI Protein Information: calsequestrin-2; calsequestrin, cardiac muscle isoform; calsequestrin 2, fast-twitch, cardiac muscle
• UniProt Protein Name: Calsequestrin-2
• Protein Family: Calsequestrin
• UniProt Gene Name: CASQ2
• UniProt Entry Name: CASQ2_HUMAN

NCBI Description

The protein encoded by this gene specifies the cardiac muscle family member of the calsequestrin family. Calsequestrin is localized to the sarcoplasmic reticulum in cardiac and slow skeletal muscle cells. The protein is a calcium binding protein that stores calcium for muscle function. Mutations in this gene cause stress-induced polymorphic ventricular tachycardia, also referred to as catecholaminergic polymorphic ventricular tachycardia 2 (CPVT2), a disease characterized by bidirectional ventricular tachycardia that may lead to cardiac arrest. [provided by RefSeq, Jul 2008]

Uniprot Description

calsequestrin 2: Calsequestrin is a high-capacity, moderate affinity, calcium-binding protein and thus acts as an internal calcium store in muscle. The release of calcium bound to calsequestrin through a calcium release channel triggers muscle contraction. The skeletal muscle isoform (CASQ1) binds around 80 Ca(2+) ions, while the cardiac isoform (CASQ2) binds approximately 60 Ca(2+) ions. Defects in CASQ2 are the cause of catecholaminergic polymorphic ventricular tachycardia type 2 (CPVT2); also known as stress-induced polymorphic ventricular tachycardia (VTSIP). CPVT2 is an autosomal recessive form of arrhythmogenic disorder characterized by stress-induced, bidirectional ventricular tachycardia that may degenerate into cardiac arrest and cause sudden death. Belongs to the calsequestrin family.

Protein type: Calcium-binding

Chromosomal Location of Human Ortholog: 1p13.1

Cellular Component: sarcoplasmic reticulum membrane; sarcoplasmic reticulum lumen; sarcoplasmic reticulum; cytoplasm; junctional membrane complex; voltage-gated calcium channel complex; intracellular; Z disc

Molecular Function: protein binding; protein homodimerization activity; calcium ion binding; calcium-dependent protein binding

Biological Process: protein polymerization; striated muscle contraction; regulation of heart rate; sequestering of calcium ion; negative regulation of potassium ion transport; detection of calcium ion; sarcomere organization; transmembrane transport; cardiac muscle contraction

Disease: Ventricular Tachycardia, Catecholaminergic Polymorphic, 1, With Or Without Atrial Dysfunction And/or Dilated Cardiomyopathy; Ventricular Tachycardia, Catecholaminergic Polymorphic, 2