產(chǎn)品編號 yb-11708R
英文名稱 Anti-ATP13A2抗體
中文名稱 帕金森病相關(guān)蛋白ATP13A2抗體
別    名 PARK9; AT132_HUMAN; Atp13a2; ATPase type 13A2; CLN12; HSA9947; KRPPD; PARK9; Probable cation transporting ATPase 13A2; Probable cation-transporting ATPase 13A2; Putative ATPase; RP1-37C10.4.
 Anti-ATP13A2抗體   
說 明 書 0.2ml  
研究領(lǐng)域 腫瘤  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  細(xì)胞膜蛋白  Alzheimer's  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, 
產(chǎn)品應(yīng)用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 129kDa
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ATP13A2 (1001-1080aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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(chǎn)品介紹 background:
ATP13A2 is a 1,180 amino acid multi-pass membrane protein that belongs to the P5 subfamily of ATPases which play an important role in the transportation of inorganic cations. Expressed as multiple alternative spliced isoforms, ATP13A2 functions to catalyze the conversion of ATP to ADP and a free phosphate, thereby participating in the active transport of ions across cellular membranes. Defects in the gene encoding ATP13A2 are the cause of Kufor-Rakeb syndrome (KRS), a rare hereditary type of Parkinson’s disease that exhibits juvenile onset and is characterized by neurodegeneration and dementia. The ATP13A2 gene maps to human chromosome 1, which spans 260 million base pairs, contains over 3,000 genes and comprises nearly 8% of the human genome.

Function:
May play a role in intracellular cation homeostasis and the maintenance of neuronal integrity. 

Subcellular Location:
Membrane; Multi-pass membrane protein (By similarity). Lysosome. 

Tissue Specificity:
Expressed in brain; protein levels are markedly increased in brain from subjects with Parkinson disease and subjects with dementia with Lewy bodies. Detected in pyramidal neurons located throughout the cingulate cortex (at protein level). In the substantia nigra, it is found in neuromelanin-positive dopaminergic neurons (at protein level). 

DISEASE:
Defects in ATP13A2 are the cause of Kufor-Rakeb syndrome (KRS) [MIM:606693]; also known as Parkinson disease type 9 (PARK9). KRS is a rare hereditary disease with juvenile onset. In addition to typical signs of Parkinson disease, affected individuals show symptoms of more widespread neurodegeneration, including dementia.

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

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