英文名称
PARK7 | DJ1 Rabbit pAb
英文别名
DJ-1; DJ1; GATD2; HEL-S-67p; CAP1; SP22; PARK7_BOVIN; PARK7; Maillard deglycase; Parkinsonism-associated deglycase; Protein DJ-1 (DJ-1); Protein/nucleic acid deglycase DJ-1; PARK7_HUMAN; Oncogene DJ1; PARK7_MOUSE; PARK7_RAT; Contraception-associated protein 1 (Protein CAP1); Fertility protein SP22; Parkinsonism associated deglycase; Parkinson disease (autosomal recessive, early onset) 7; parkinson protein 7
免疫原
KLH conjugated synthetic peptide derived from human CAP1: 101-189/189
纯化方法
affinity purified by Protein A
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍
DJ-1蛋白是近年来新发现的一种肿瘤蛋白,DJ-1能有效保护细胞抵抗氧化应激, 该蛋白早期主要用于肿瘤方面的研究,近年来科研人员经研究认为:DJ-1蛋白与神经退化及损伤有一定的关联。
背景资料
PARK7/DJ1 is a ubiquitously expressed protein involved in various cellular processes including cell proliferation, RNA-binding, and oxidative stress. The protein has been found to colocalize within a subset of pathologic tau inclusions in a diverse group of neurodegenerative disorders known as tauopathies (Rizzu et al. 2004). Defects in PARK7/DJ1 are the cause of autosomal recessive early-onset Parkinson's disease 7 (PARK7). Parkinson's disease (PD) is a complex, multifactorial disorder that typically manifests after the age of 50 years. The disease is characterized by bradykinesia, resting tremor, muscular rigidity and postural instability. The pathology involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain. PARK7 is characterized by onset before 40 years and slow progression. It has also been suggested that PARK7/DJ1 is a mitogen dependent oncogene product involved in Ras related signal transduction pathways.
蛋白名
Parkinson disease protein 7
亚细胞定位
Cytoplasm. Nucleus. Mitochondrion. Under normal conditions, located predominantly in the cytoplasm and, to a lesser extent, in the nucleus and mitochondrion. Translocates to the mitochondrion and subsequently to the nucleus in response to oxidative stress and exerts an increased cytoprotective effect against oxidative damage. Detected in tau inclusions in brains from neurodegenerative disease patients.
组织特异性
Highly expressed in pancreas, kidney, skeletal muscle, liver, testis and heart. Detected at slightly lower levels in placenta and brain. Detected in astrocytes, Sertoli cells, spermatogonia, spermatids and spermatozoa.
翻译后修饰
Sumoylated on Lys-130 by PIAS2 or PIAS4; which is enhanced after ultraviolet irradiation and essential for cell-growth promoting activity and transforming activity.
疾病
Defects in PARK7 are the cause of Parkinson disease type 7 (PARK7) [MIM:606324]. A neurodegenerative disorder characterized by resting tremor, postural tremor, bradykinesia, muscular rigidity, anxiety and psychotic episodes. PARK7 has onset before 40 years, slow progression and initial good response to levodopa. Some patients may show traits reminiscent of amyotrophic lateral sclerosis-parkinsonism/dementia complex (Guam disease).
相似性
Belongs to the peptidase C56 family.
功能
Protects cells against oxidative stress and cell death. Plays a role in regulating expression or stability of the mitochondrial uncoupling proteins SLC25A14 and SLC25A27 in dopaminergic neurons of the substantia nigra pars compacta and attenuates the oxidative stress induced by calcium entry into the neurons via L-type channels during pacemaking. Eliminates hydrogen peroxide and protects cells against hydrogen peroxide-induced cell death. May act as an atypical peroxiredoxin-like peroxidase that scavenges hydrogen peroxide. Following removal of a C-terminal peptide, displays protease activity and enhanced cytoprotective action against oxidative stress-induced apoptosis. Stabilizes NFE2L2 by preventing its association with KEAP1 and its subsequent ubiquitination. Binds to OTUD7B and inhibits its deubiquitinating activity. Enhances RELA nuclear translocation. Binds to a number of mRNAs containing multiple copies of GG or CC motifs and partially inhibits their translation but dissociates following oxidative stress. Required for correct mitochondrial morphology and function and for autophagy of dysfunctional mitochondria. Regulates astrocyte inflammatory responses. Acts as a positive regulator of androgen receptor-dependent transcription. Prevents aggregation of SNCA. Plays a role in fertilization. Has no proteolytic activity. Has cell-growth promoting activity and transforming activity. May function as a redox-sensitive chaperone.