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Rabbit Anti-PARK7/DJ1/FITC Conjugated antibody
background:
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.
Function:
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 SLCterminal 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.
Subcellular Location:
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.
Tissue Specificity:
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.
Post-translational modifications:
Sumoylated on Lys-130 by PIAS2 or PIAS4; which is enhanced after ultraviolet irradiation and essential for cell-growth promoting activity and transforming activity.
DISEASE:
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).
Similarity:
Belongs to the peptidase C56 family.
Database links:
Entrez Gene: 11315 Human
Entrez Gene: 5764 Mouse
Entrez Gene: 117287 Rat
Entrez Gene: 511268 Cow
Entrez Gene: 479595 Dog
Omim: 602533 Human
SwissProt: Q5E946 Cow
SwissProt: Q99497 Human
SwissProt: Q99LX0 Mouse
SwissProt: O88767 Rat
Unigene: 419128 Human
Unigene: 277349 Mouse
Unigene: 30105 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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。
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