LRRK2 (D18E12) Rabbit mAb

• 产品编号：CST-13046S      品牌：CST       原厂货号：13046S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

LRRK2 (D18E12) Rabbit mAb 兔单抗可以识别内源性的LRRK2总蛋白。该单克隆抗体由合成的人LRRK2蛋白Pro2080邻近肽段免疫动物而产生。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）（1）。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）。富含亮氨酸重复的激酶2（LRRK2）在其氨基末端富含亮氨酸重复序列（LRR），含有一个RAS样小GTP结合蛋白样（ROC）结构域，一个MLK蛋白激酶域和一个羧基末端的WD40结构重复域。至少20个LRRK2基因突变与帕金森病G2019S突变有普遍的联系（3）。G2019S突变导致增加的LRRK2激酶活性，导致神经突起长度逐步减少，以及进行性的神经轴突损失和神经细胞的生存数目减少（4）。MLK阻断剂CEP-1347正在进行帕金森病的临床试验，结果表明LRRK2基因可能作为治疗PD的潜在靶点（5）。

1. Fahn, S. (2003) Ann. NY Acad. Sci. 991, 1-14.
2. Moore, D.J. et al. (2005) Annu. Rev. Neurosci. 28, 57-87.
3. Mata, I.F. et al. (2006) Trends Neurosci. 29, 286-293.
4. MacLeod, D. et al. (2006) Neuron 52, 587-593.
5. Parkinson Study Group. (2004) Neurology 62, 330-332.

原厂资料：

Specificity / Sensitivity

LRRK2 (D18E12) recognizes endogenous levels of total LRRK2 protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Pro2080 of human LRRK2 protein.

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 hallmarks of PD are 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). Research studies have shown various genes and loci are genetically linked to PD including α-synuclein/PARK1 and 4, parkin/PARK2, UCH-L1/PARK5, PINK1/PARK6, DJ-1/PARK7, LRRK2/PARK8, synphilin-1, and NR4A2 (2).

Leucine-rich repeat kinase 2 (LRRK2) contains amino-terminal leucine-rich repeats (LRR), a Ras-like small GTP binding protein-like (ROC) domain, an MLK protein kinase domain, and a carboxy-terminal WD40 repeat domain. Research studies have linked at least 20 LRRK2 mutations to PD, with the G2019S mutation being the most prevalent (3). The G2019S mutation causes increased LRRK2 kinase activity, which induces a progressive reduction in neurite length that leads to progressive neurite loss and decreased neuronal survival (4). Researchers are currently testing the MLK inhibitor CEP-1347 in PD clinical trials, indicating the potential value of LRRK2 as a therapeutic target for treatment of PD (5).

1. Fahn, S. (2003) Ann. NY Acad. Sci. 991, 1-14.
2. Moore, D.J. et al. (2005) Annu. Rev. Neurosci. 28, 57-87.
3. Mata, I.F. et al. (2006) Trends Neurosci. 29, 286-293.
4. MacLeod, D. et al. (2006) Neuron 52, 587-593.
5. Parkinson Study Group. (2004) Neurology 62, 330-332.

注意事项：

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150
mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02%
sodium azide. Store at –20°C. Do not aliquot the antibody.