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Mouse Anti-CASQ1 Recombinant Antibody (CBLC119-LY) (CBMAB-C9752-LY)


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Published Data
CBMAB-C9752-LY-1
Mouse Anti-CASQ1 Recombinant Antibody (CBLC119-LY) (CBMAB-C9752-LY) in Immunoblot
Immunoblot analyses of RyR, DHPR, JP1, and CSQ1 in tibialis anterior muscles. RyR, DHPR, and JP1 were analyzed on the same membrane. For RyR and DHPR, in addition to full-length proteins, faster migrating bands were observed and most likely corresponded to degraded proteins. ...View More
Kanzaki, K., Watanabe, D., Aibara, C., Kawakami, Y., Yamada, T., Takahashi, Y., & Wada, M. (2018). l-arginine ingestion inhibits eccentric contraction-induced proteolysis and force deficit via S-nitrosylation of calpain. Physiological reports, 6(2), e13582.

Summary

Host Animal
Mouse
Specificity
Rabbit, Human, Mouse, Rat, Dog
Clone
CBLC119-LY
Antibody Isotype
IgG2b, κ
Application
WB, IP

Basic Information

Immunogen
Purified rabbit skeletal muscle sarcoplasmic reticulum.
Host Species
Mouse
Specificity
Rabbit, Human, Mouse, Rat, Dog
Antibody Isotype
IgG2b, κ
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:100-1:1,000
IP1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, 0.1% gelatin
Preservative
< 0.1% sodium azide
Concentration
0.2 mg/ml
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
calsequestrin 1 (fast-twitch, skeletal muscle)
Introduction
CASQ1 (Calsequestrin 1) is a Protein Coding gene. Diseases associated with CASQ1 include Myopathy, Vacuolar, With Casq1 Aggregates and Myopathy, Tubular Aggregate, 1. Among its related pathways are Antiarrhythmic Pathway, Pharmacodynamics and Myometrial Relaxation and Contraction Pathways. Gene Ontology (GO) annotations related to this gene include calcium ion binding. An important paralog of this gene is CASQ2.
Entrez Gene ID
100009095
UniProt ID
P07221
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 Ca2+ ions (PubMed:28895244).
Regulates the release of lumenal Ca2+ via the calcium release channel RYR1; this plays an important role in triggering muscle contraction. Negatively regulates store-operated Ca2+ entry (SOCE) activity (PubMed:27185316).
Biological Process
Endoplasmic reticulum organization Source: Ensembl
Ion transmembrane transport Source: Reactome
Positive regulation of release of sequestered calcium ion into cytosol Source: UniProtKB
Positive regulation of store-operated calcium channel activity Source: UniProtKB
Protein polymerization Source: UniProtKB
Regulation of cardiac conduction Source: Reactome
Regulation of release of sequestered calcium ion into cytosol Source: GO_Central
Regulation of skeletal muscle contraction by regulation of release of sequestered calcium ion Source: UniProtKB
Regulation of store-operated calcium entry Source: UniProtKB
Response to denervation involved in regulation of muscle adaptation Source: Ensembl
Response to heat Source: Ensembl
Response to organic substance Source: Ensembl
Sarcomere organization Source: UniProtKB
Skeletal muscle tissue development Source: Ensembl
Cellular Location
Endoplasmic reticulum; Mitochondrion matrix; Sarcoplasmic reticulum; Sarcoplasmic reticulum lumen; Sarcoplasmic reticulum membrane. 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).
Involvement in disease
Myopathy, vacuolar, with CASQ1 aggregates (VMCQA): An autosomal dominant mild muscle disorder characterized by adult 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 (TAM1): 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.
PTM
N-glycosylated.
More Infomation

Sun, Z., Wang, L., Han, L., Wang, Y., Zhou, Y., Li, Q., ... & Luo, D. (2021). Functional Calsequestrin-1 Is Expressed in the Heart and Its Deficiency Is Causally Related to Malignant Hyperthermia-Like Arrhythmia. Circulation, 144(10), 788-804.

Jeong, S. Y., Oh, M. R., Choi, J. H., Woo, J. S., & Lee, E. H. (2021). Calsequestrin 1 Is an Active Partner of Stromal Interaction Molecule 2 in Skeletal Muscle. Cells, 10(11), 2821.

Rossi, D., Gamberucci, A., Pierantozzi, E., Amato, C., Migliore, L., & Sorrentino, V. (2021). Calsequestrin, a key protein in striated muscle health and disease. Journal of Muscle Research and Cell Motility, 42(2), 267-279.

Michelucci, A., Boncompagni, S., Pietrangelo, L., Takano, T., Protasi, F., & Dirksen, R. T. (2020). Pre-assembled Ca2+ entry units and constitutively active Ca2+ entry in skeletal muscle of calsequestrin-1 knockout mice. Journal of General Physiology, 152(10), e202012617.

Guarnier, F. A., Michelucci, A., Serano, M., Pietrangelo, L., Pecorai, C., Boncompagni, S., & Protasi, F. (2018). Aerobic training prevents heatstrokes in calsequestrin-1 knockout mice by reducing oxidative stress. Oxidative medicine and cellular longevity, 2018.

Böhm, J., Lornage, X., Chevessier, F., Birck, C., Zanotti, S., Cudia, P., ... & Laporte, J. (2018). CASQ1 mutations impair calsequestrin polymerization and cause tubular aggregate myopathy. Acta neuropathologica, 135(1), 149-151.

Michelucci, A., Boncompagni, S., Canato, M., Reggiani, C., & Protasi, F. (2017). Estrogens protect calsequestrin-1 knockout mice from lethal hyperthermic episodes by reducing oxidative stress in muscle. Oxidative medicine and cellular longevity, 2017.

Böhm, J., Lornage, X., Zanotti, S., Cudia, P., Schneider-Gold, C., Malfatti, E., ... & Laporte, J. (2017). CASQ1 mutations impair calsequestrin polymerization and cause tubular aggregate myopathy. Neuromuscular Disorders, 27, S176.

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For research use only. Not intended for any clinical use.

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