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ATP13A2 Rabbit pAb (bs-11708R)  
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產(chǎn)品編號(hào) bs-11708R
英文名稱 ATP13A2 Rabbit pAb
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.  
研究領(lǐng)域 腫瘤  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞膜蛋白  Alzheimer's  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號(hào)
交叉反應(yīng) Rat (predicted: Human,Mouse,Rabbit,Pig,Cow,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 129 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ATP13A2: 1001-1080/1180 <Extracellular>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
Q9NQ11

Gene ID:
23400

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




產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ATP13A2) Polyclonal Antibody, Unconjugated (bs-11708R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.
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