Calcium-transporting ATPase type 2C member 1, ATP2C1A, HHD, BCPM, PMR1, SPCA1, hSPCA1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

At least four isoforms of ATP2C1 are known to exist; this antibody will recognize only the three longest isoforms. ATP2C1 antibody will not cross-react with ATP2C2.

Affinity chromatography purified via peptide column

Supplied in PBS containing 0.02% sodium azide.

Can be stored at -20 degree C, stable for one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Small volumes of anti-ATP2C1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ATP2C1, also known as secretory pathway Ca2+/Mn2+-ATPase (SPCA) 1, belongs to the family of P-type cation transport ATPases. This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of the calcium from the cytosol to the Golgi lumen. Defects in this gene cause Hailey-Hailey disease, an autosomal dominant disorder characterized by persistent blisters and erosions of the skin. Unlike the related protein ATP2C2, ATP2C1 is ubiquitously expressed and displays a lower maximal turnover rate for overall Ca2+-ATPase reaction and a higher apparent affinity for cytosolic Ca2+ activation of phosphorylation. Recent evidence suggests that ATP2C1 is a key regulator of insulin-like growth factor receptor (IGF1R) processing in tumor progression in basal breast cancers.

Total protein Ab

ELISA (EIA), Western Blot (WB)

Western blot analysis of ATP2C1 in mouse brain tissue lysate with ATP2C1 antibody at 1 ug/mL in (A) the absence and (B) the presence of blocking peptide.

104,032 Da

ATPase, Ca++ transporting, type 2C, member 1

calcium-transporting ATPase type 2C member 1

Calcium-transporting ATPase type 2C member 1

KIAA1347; PMR1L; ATPase 2C1??[Similar Products]

AT2C1_HUMAN

The protein encoded by this gene belongs to the family of P-type cation transport ATPases. This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of calcium ions. Defects in this gene cause Hailey-Hailey disease, an autosomal dominant disorder. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Aug 2011]

ATP2C1: This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of the calcium. Defects in ATP2C1 are the cause of Hailey-Hailey disease (HHD); also known as benign familial pemphigus. HHD is an autosomal dominant disorder characterized by persistent blisters and suprabasal cell separation (acantholysis) of the epidermis, due to impaired keratinocyte adhesion. Patients lacking all isoforms except isoform 2 have HHD. Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IIA subfamily. 6 isoforms of the human protein are produced by alternative splicing.

Protein type: Transporter, ion channel; Membrane protein, multi-pass; Hydrolase; Membrane protein, integral; Transporter; EC 3.6.3.8

Chromosomal Location of Human Ortholog: 3q22.1

Cellular Component: Golgi membrane; Golgi apparatus; membrane; integral to membrane; trans-Golgi network

Molecular Function: manganese-transporting ATPase activity; signal transducer activity; calcium-transporting ATPase activity; metal ion binding; manganese ion binding; calcium ion binding; ATP binding

Biological Process: cellular calcium ion homeostasis; Golgi calcium ion homeostasis; epidermis development; positive regulation of I-kappaB kinase/NF-kappaB cascade; metabolic process; calcium ion transport; actin cytoskeleton reorganization; calcium-dependent cell-cell adhesion; manganese ion transport; cellular manganese ion homeostasis; signal transduction; Golgi calcium ion transport; transmembrane transport

Disease: Benign Chronic Pemphigus

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