产品名称：Anti-ATP13A2/FITC抗体

Anti-ATP13A2/FITC抗体

产品编号YB11708R-FITC

英文名称Anti-ATP13A2/FITC

中文名称FITC标记的帕金森病相关蛋白ATP13A2抗体

别    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/FITC抗体

说 明 书100ul  

研究领域肿瘤  神经生物学  信号转导  细胞膜蛋白  Alzheimer's  

抗体来源Rabbit

克隆类型Polyclonal

交叉反应Human, Mouse, Rat, Pig, Cow, Horse, Rabbit,

产品应用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

细胞定位细胞膜

性    状Lyophilized or Liquid

浓    度1mg/ml

免 疫 原KLH conjugated synthetic peptide derived from human ATP13A2 (1001-1080aa)

亚    型IgG

纯化方法affinity purified by Protein A

储 存 液Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

保存条件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.

Anti-ATP13A2/FITC抗体

产品介绍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 levelsAnti-ATP13A2/FITC抗体 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.

Anti-ATP13A2/FITC抗体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.