產品編號 YB-2434R
英文名稱 Anti-ATP2c1抗體
中文名稱 鈣離子ATP酶通道蛋白抗體
別    名 1700121J11Rik; AT2C1_HUMAN; ATP dependent Ca(2+) pump PMR1; ATP-dependent Ca(2+) pump PMR1; ATP2C1; ATPase 2C1; ATPase Ca(2+) sequestering; ATPase Ca++ transporting type 2C member 1; AW061228; BCPM; Calcium transporting ATPase type 2C member 1; Calcium-transporting ATPase type 2C member 1; D930003G21Rik; HHD; hSPCA1; HUSSY 28; KIAA1347; MGC58010; MGC93231; OTTHUMP00000216066; OTTHUMP00000216068; OTTHUMP00000216069; OTTHUMP00000216071; OTTHUMP00000216072; OTTHUMP00000216073; OTTHUMP00000216074; OTTHUMP00000216075; PMR1; PMR1L; Secretory pathway Ca(2+)-transporting ATPase; Secretory pathway Ca2+/Mn2+ ATPase 1; SPCA; SPCA1.
Anti-ATP2c1抗體
說 明 書 0.1ml  0.2ml  
研究領域 免疫學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, 
產品應用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 101kDa
細胞定位 細胞漿 細胞膜 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ATP2c1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
產品介紹 background:
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]

Function:
This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of the calcium.

Subcellular Location:
Golgi apparatus membrane; Multi-pass membrane protein.

Tissue Specificity:
Found in most tissues except colon, thymus, spleen and leukocytes. Most abundant in keratinocytes and kidney. 

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.

Similarity:
Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IIA subfamily.


Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.