ATP2C1A; BCPM; HHD; PMR1; SPCA1; hSPCA1; Benign Chronic Pemphigus(Hailey-Hailey Disease); Secretory Pathway Ca2+/Mn2+ ATPase 1

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

Host: E Coli
Source: Prokaryotic Expression

> 90%

Freeze-Dried Powder; PBS, pH7.4, Containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.

Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months. Avoid repeated freeze/thaw cycles.
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of ATP2C1 recombinant protein 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.

Signal Transduction

Positive Control, Immunogen, SDS-PAGE, Western Blot (WB)

Predicted MW: 11.6kDa
Accurate MW: 15kDa

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]

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; Hydrolase; Membrane protein, multi-pass; Membrane protein, integral; Transporter, ion channel; 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; manganese ion binding; metal 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; calcium-dependent cell-cell adhesion; actin cytoskeleton reorganization; manganese ion transport; cellular manganese ion homeostasis; signal transduction; Golgi calcium ion transport; transmembrane transport

Disease: Benign Chronic Pemphigus

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