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Rabbit Anti-ATP13A2/BF594 Conjugated antibody (bs-11708R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11708R-BF594
英文名稱 Rabbit Anti-ATP13A2/BF594 Conjugated antibody
中文名稱 BF594標記的帕金森病相關蛋白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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  神經生物學  信號轉導  細胞膜蛋白  Alzheimer's  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 129kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
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 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.
產品介紹 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.

Database links:

Entrez Gene: 23400 Human

Entrez Gene: 74772 Mouse

Entrez Gene: 362645 Rat

SwissProt: Q9NQ11 Human

SwissProt: Q9CTG6 Mouse

Unigene: 128866 Human

Unigene: 205625 Mouse

Unigene: 19659 Rat

Omim: 610513 Human




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