ITM0524
ITM0524
- Catalog: ITM0524
- Gene/Protein: ATP2C1
- Product Description: Immunotag™ PMR1 Monoclonal Antibody
504.0000
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Antibody Specification | |
Datasheet | |
Target Protein | PMR1 |
Clonality | Monoclonal |
Storage/Stability | -20°C/1 year |
Application | WB,IHC-p,ELISA |
Recommended Dilution | Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/200 - 1/1000. ELISA: 1/10000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human,Monkey |
Host Species | Mouse |
Immunogen | Purified recombinant fragment of PMR1 expressed in E. Coli. |
Specificity | PMR1 Monoclonal Antibody detects endogenous levels of PMR1 protein. |
Purification | Affinity purification |
Form | Ascitic fluid containing 0.03% sodium azide. |
Gene Name | ATP2C1 |
Accession No. | P98194 Q80XR2 |
Alternate Names | ATP2C1; KIAA1347; PMR1L; HUSSY-28; Calcium-transporting ATPase type 2C member 1; ATPase 2C1; ATP-dependent Ca(2+) pump PMR1 |
Description | ATPase secretory pathway Ca2+ transporting 1(ATP2C1) Homo sapiens 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], |
Protein Expression | Blood,Brain,Keratinocyte,Mammary gland,Neuron, |
Subcellular Localization | Golgi membrane,Golgi apparatus,trans-Golgi network,integral component of plasma membrane,membrane,integral component of membrane, |
Protein Function | Isoform 1 and isoform 2 are expressed in the same tissues,catalytic activity:ATP + H(2)O + Ca(2+)(Cis) = ADP + phosphate + Ca(2+)(Trans).,disease:Defects in ATP2C1 are the cause of Hailey-Hailey disease (HHD) [MIM:169600]; 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.,function:This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of the calcium.,similarity:Belongs to the cation transport ATPase (P-type) family.,similarity:Belongs to the cation transport ATPase (P-type) family. Type IIA subfamily.,tissue specificity:Found in most tissues except colon, thymus, spleen and leukocytes. Most abundant in keratinocytes and kidney., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |