英文名称
PGP9.5 Recombinant Rabbit mAb
英文别名
HEL-117; HEL-S-53; NDGOA; PARK5; PGP 9.5; PGP9.5; PGP95; SPG79; SPG79A; UCHL-1; Uch-L1; gad; UCHL1_HUMAN; UCHL1; Neuron cytoplasmic protein 9.5; PGP 9.5 (PGP9.5); Ubiquitin thioesterase L1; 3.4.19.12; UCHL1_MOUSE; UCHL1_PIG; UCHL1_RAT; ubiquitin C-terminal hydrolase L1; ubiquitin carboxyl-terminal esterase L1 (ubiquitin thiolesterase); ubiquitin thiolesterase
免疫原
Recombinant human UCHL1 protein
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 °C 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
该抗原是一种神经特异性蛋白,广泛分布于中枢与外周神经系统的神经元和神经纤维、神经内分泌细胞、肾小管段、睾丸精原细胞、Leydig细胞、卵细胞以及妊娠与非妊娠黄体内的某些细胞。用于标记神经元,对研究几种人慢性神经变性性疾病中广泛存在的细胞包涵体特征较有意义。
背景资料
catalyzes the hydrolysis of ubiquitin carboxy-terminal thiolesters to form ubiquitin and a thiol; may play a role in neuropathic pain [RGD].
Found in neuronal cell bodies and processes throughout the neocortex (at protein level). Expressed in neurons and cells of the diffuse neuroendocrine system and their tumors. Weakly expressed in ovary. Down-regulated in brains from Parkinson disease and Alzheimer disease patients.
蛋白名
Ubiquitin carboxyl-terminal hydrolase isozyme L1
亚基
Monomer. Homodimer. Interacts with SNCA (By similarity). Interacts with COPS5.
亚细胞定位
Cytoplasm. Endoplasmic reticulum membrane. About 30% of total UCHL1 is associated with membranes in brain.
组织特异性
Found in neuronal cell bodies and processes throughout the neocortex (at protein level). Expressed in neurons and cells of the diffuse neuroendocrine system and their tumors. Weakly expressed in ovary. Down-regulated in brains from Parkinson disease and Alzheimer disease patients.
疾病
Defects in UCHL1 are the cause of Parkinson disease type 5 (PARK5) [MIM:613643]; also known as Parkinson disease autosomal dominant 5. PARK5 is a complex neurodegenerative disorder with manifestations ranging from typical Parkinson disease to dementia with Lewy bodies. Clinical features include parkinsonian symptoms (resting tremor, rigidity, postural instability and bradykinesia), dementia, diffuse Lewy body pathology, autonomic dysfunction, hallucinations and paranoia.
相似性
Belongs to the peptidase C12 family.
功能
Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. This enzyme is a thiol protease that recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. Also binds to free monoubiquitin and may prevent its degradation in lysosomes. The homodimer may have ATP-independent ubiquitin ligase activity.