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A3361

Sigma-Aldrich

Anti-ATP13A2 (C-terminal region) antibody produced in rabbit

enhanced validation

~1.5 mg/mL, affinity isolated antibody, buffered aqueous solution

同義詞:

Anti-ATPase type 13A2, Anti-PARK9

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About This Item

分類程式碼代碼:
12352203
NACRES:
NA.41

生物源

rabbit

共軛

unconjugated

抗體表格

affinity isolated antibody

抗體產品種類

primary antibodies

無性繁殖

polyclonal

形狀

buffered aqueous solution

分子量

antigen ~129 kDa

物種活性

mouse, human

加強驗證

recombinant expression
Learn more about Antibody Enhanced Validation

濃度

~1.5 mg/mL

技術

western blot: 1.5-3.0 μg/mL using mouse brain extract (S1 fraction) or HEK-293T cells expressing human ATP13A2

UniProt登錄號

運輸包裝

dry ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

human ... ATP13A2(23400)

一般說明

ATP13A2 (ATPase type 13A2, also known as PARK9) is a neuronal P-type ATPase of the P5 subfamily. It is present in the lysosome of transiently transfected cells, whereas the unstable truncated mutants are retained in the endoplasmic reticulum and degraded by the proteasome.
ATP13A2 is a member of the P5 subfamily of P-type transport ATPases which include ATP13A1-ATP13A5. Mutations in ATP3A2 also known as PARK9 are associated with hereditary Parkinson′s disease.
Rabbit anti-ATP13A2 (C-terminal region) antibody is specific for human and mouse ATP13A2. Staining of the ATP13A2 band by immunoblotting is specifically inhibited by the ATP13A2 immunizing peptide.

應用

Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Western Blotting (1 paper)
Rabbit anti-ATP13A2 (C-terminal region) antibody has been used for western blotting applications at a dilution of 1:1000.

生化/生理作用

ATP13A2 (ATPase type 13A2, also known as PARK9) shows elevated expression levels in the brains of sporadic Parkinson′s disease (PD) patients, suggesting a potential role in the more common forms of PD. It is associated with Kufor-Rakeb syndrome (KRS). KRS is a rare form of hereditary PD with juvenile onset. In addition to typical signs of PD, affected individuals show symptoms of more widespread pyramidal neurodegeneration, including dementia.

外觀

Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

免責聲明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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儲存類別代碼

12 - Non Combustible Liquids

水污染物質分類(WGK)

nwg


分析證明 (COA)

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Hereditary parkinsonism with dementia is caused by mutations in ATP13A2, encoding a lysosomal type 5 P-type ATPase
Ramirez A, et al.
Nature Genetics, 38(10), 1184-1184 (2006)
Alejandro Estrada-Cuzcano et al.
Brain : a journal of neurology, 140(2), 287-305 (2017-02-01)
Hereditary spastic paraplegias are heterogeneous neurodegenerative disorders characterized by progressive spasticity of the lower limbs due to degeneration of the corticospinal motor neurons. In a Bulgarian family with three siblings affected by complicated hereditary spastic paraplegia, we performed whole exome
David Ramonet et al.
Human molecular genetics, 21(8), 1725-1743 (2011-12-22)
Mutations in the ATP13A2 gene (PARK9, OMIM 610513) cause autosomal recessive, juvenile-onset Kufor-Rakeb syndrome and early-onset parkinsonism. ATP13A2 is an uncharacterized protein belonging to the P(5)-type ATPase subfamily that is predicted to regulate the membrane transport of cations. The physiological
Mark Ainsley Colijn et al.
Neurogenetics (2024-07-18)
Biallelic (autosomal recessive) pathogenic variants in ATP13A2 cause a form of juvenile-onset parkinsonism, termed Kufor-Rakeb syndrome. In addition to motor symptoms, a variety of other neurological and psychiatric symptoms may occur in affected individuals, including supranuclear gaze palsy and cognitive
Shaun Martin et al.
Parkinson's disease, 2016, 9531917-9531917 (2016-04-14)
The late endo-/lysosomal P-type ATPase ATP13A2 (PARK9) is implicated in Parkinson's disease (PD) and Kufor-Rakeb syndrome, early-onset atypical Parkinsonism. ATP13A2 interacts at the N-terminus with the signaling lipids phosphatidic acid (PA) and phosphatidylinositol (3,5) bisphosphate (PI(3,5)P2), which modulate ATP13A2 activity

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