PARK9 Antibody

• 产品编号：CST-5879S      品牌：CST       原厂货号：5879S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

PARK9 Antibody 兔多抗识别内源性的总PARK9蛋白。该多克隆抗体由合成的人类PARK9中Ser282位点附近肽段免疫动物而制成。该抗体使用蛋白A和多肽亲和层析纯化而得。帕金森病（PD），是除阿尔茨海默氏病外的第二个最常见的神经退行性疾病，是一种以强直，震颤和姿势不稳为特点的进行性的运动障碍。帕金森病的病理特点是黑质及腹侧中脑的多巴胺能神经元逐渐丧失，并在尚存活的细胞内出现Lewy小体（α-synuclein，泛素和其他相关成分的堆积）。不同的基因及其位点（α-synuclein/PARK1和4，parkin/PARK2，UCH-L1/PARK5，PINK1/PARK6，DJ-1/PARK7，LRRK2/PARK8，ATP13A2/PARK9）都与PD相关（2）。PARK9，也称为ATP13A2，是P-型ATP酶超家族的成员，并与溶酶体降解途径和α-突触核蛋白堆积物清除有关（3,4）。该蛋白有10个跨膜结构域，野生型PARK9定位于溶酶体膜。相反，所有三个已知的突变，导致过早出血终止密码子而截断蛋白，这些蛋白被保留在内质网和蛋白酶体中降解。PARK9主要表达于大脑中，并与这Kufor-Rakeb综合症，一种单基因隐性遗传的，非典型的帕金森综合征有关，这种疾病以少年发病，锥体变性和认知功能障碍为特点（5,6）。

1. Fahn, S. (2003) Ann N Y Acad Sci 991, 1-14.
2. Moore, D.J. et al. (2005) Annu Rev Neurosci 28, 57-87.
3. Ramirez, A. et al. (2006) Nat Genet 38, 1184-91.
4. Xiromerisiou, G. et al. (2010) Neurosurg Focus 28, E7.
5. Klein, C. and Lohmann-Hedrich, K. (2007) Curr Opin Neurol 20, 453-64.

原厂资料：

Specificity / Sensitivity

PARK9 Antibody recognizes endogenous levels of total PARK9 protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Ser282 of human PARK9 protein. Antibodies are purified by protein A and peptide affinity chromatography.

Background

Parkinson’s disease (PD), the second most common neurodegenerative disease after Alzheimer’s, is a progressive movement disorder characterized by rigidity, tremors and postural instability. The pathological hallmark of PD is progressive loss of dopaminergic neurons in the substantia nigra of the ventral midbrain and the presence of intracellular Lewy bodies (protein aggregates of α-synuclein, ubiquitin and other components) in surviving neurons of the brain stem (1). Various genes and loci (α-synuclein/PARK1 and 4, parkin/PARK2, UCH-L1/PARK5, PINK1/PARK6, DJ-1/PARK7, LRRK2/PARK8, ATP13A2/PARK9) are genetically linked to PD (2). 
 
 PARK9, also known as ATP13A2, is a member of the P-type ATPase superfamily and is involved in the lysosomal degradation pathway, clearing α-synuclein aggregates (3,4). The protein has 10 transmembrane domains and wild-type PARK9 localizes to the lysosomal membrane. In contrast, all three known mutations, which have premature stop codons resulting in a truncated protein, are retained in the endoplasmic reticulum and degraded by the proteasome. PARK9 is predominantly expressed in the brain and has been linked to Kufor-Rakeb Syndrome, a monogenic form of recessively inherited, atypical parkinsonism that is characterized by juvenile-onset, with pyramidal degeneration and cognitive dysfunction (5,6).

1. Fahn, S. (2003) Ann N Y Acad Sci 991, 1-14.
2. Moore, D.J. et al. (2005) Annu Rev Neurosci 28, 57-87.
3. Ramirez, A. et al. (2006) Nat Genet 38, 1184-91.
4. Xiromerisiou, G. et al. (2010) Neurosurg Focus 28, E7.
5. Klein, C. and Lohmann-Hedrich, K. (2007) Curr Opin Neurol 20, 453-64.

注意事项：

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM
NaCl, 100 µg/ml BSA and 50% glycerol. Store at –20°C.
Do not aliquot the antibody.