蛋白名称
Calcium-transporting ATPase type 2C member 1
免疫原
Purified recombinant fragment of PMR1 expressed in E. Coli.
特异性
PMR1 Monoclonal Antibody detects endogenous levels of PMR1 protein.
组成
Ascitic fluid containing 0.03% sodium azide,0.5% BSA, 50%glycerol.
稀释比例
WB: 1/500 - 1/2000. IHC: 1/200 - 1/1000. ELISA: 1/10000.. IF 1:50-200
纯化工艺
Affinity purification
其他名称
ATP2C1; KIAA1347; PMR1L; HUSSY-28; Calcium-transporting ATPase type 2C member 1; ATPase 2C1; ATP-dependent Ca(2+) pump PMR1
背景
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],
功能
alternative products: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 keratin
