ATP2C1, Monoclonal Antibody

HHD; BCPM; PMR1; SPCA1

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

Monoclonal

IgG1

4G12

Mouse

Ascitic fluid containing 0.03% sodium azide.

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.

Entrez Summary: ATP2C1, also known as PMR1, it 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. The human homologue, ATP2C1 (also designated SPLA in rat), also regulates the transport of calcium in the Golgi complex and is related to other P-type ATPases family members, such as the sarco (endo)plasmic calcium ATPase (SERCA) and the plasma membrane calcium ATPase (PCMA). ATP2C1 is a transmembrane protein that exists as two splice variants, which vary by 20 amino acids. Defects in ATP2C1 cause Hailey-Hailey disease, which is an autosomal dominant disorder that is characterized by blisters and erosions of the skin. These findings provide further evidence that PMR1 plays a key role in maintaining the integrity of the epidermis by controlling intracellular calcium signaling.

Western Blot (WB), Immunohistochemistry (IHC)

Western blot analysis using ATP2C1 mAb against human ATP2C1 (AA

Western blot analysis using ATP2C1 mouse mAb against A431 (1), Hela (2) and HEK293 (3) cell lysate.

Immunohistochemical analysis of paraffin-embedded human ovarian cancer (left) and breast cancer (right) tissues using ATP2C1 mouse mAb with DAB staining.

100

ATPase secretory pathway Ca2+ transporting 1

calcium-transporting ATPase type 2C member 1

Calcium-transporting ATPase type 2C member 1

KIAA1347; PMR1L; ATPase 2C1  [Similar Products]

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

Chromosomal Location of Human Ortholog: 3q22.1

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

Molecular Function: calcium-transporting ATPase activity; signal transducer activity

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

Disease: Benign Chronic Pemphigus

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