Human Recombinant CASQ1 protein (V2LY-0526-LY2523)

Name: Human Recombinant CASQ1 protein
SKU: V2LY-0526-LY2523
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Expressed Host

E. coli

Protein Species

Human

Protein Construction

This product is Human Recombinant CASQ1 protein consist of Amino Acid: 35-396 and predicts a molecular mass of 41.8 kDa.

Molecule Mass

41.8 kDa

Sequence

Amino Acid: 35-396

Species

Human

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

Purity

>80% as determined by SDS-PAGE

Endotoxin

Please contact us for more information.

Format

Lyophilized

Reconstitution

Allow the vial and reconstitution buffer to equilibrate to room temperature. Briefly centrifuge or tap down the vial to ensure that all lyophilized powder is collected at the bottom of the vial. For the reconstitution of this product, we recommend adding PBS or sterile water to achieve a final antibody concentration of 1 mg/mL. Allow the vial to reconstitute for 10-15 minutes at room temperature with gentle agitation. Avoid vigorous shaking that can cause foaming and antibody denaturation. Aliquot into volumes based on your experiment and store liquid protein at -20°C or -80°C for long time.

Buffer

Lyophilized from sterile Tris, NaCl, Glutathione, EDTA, DTT, PMSF, Glycerol

Preservative

None

Storage

Samples are stable for up to twelve months from date of receipt at -20°C to -80°C. Store it under sterile conditions at -20°C to -80°C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.

More Infomation

Target

Full Name

calsequestrin 1 (fast-twitch, skeletal muscle)

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.

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

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

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