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Mouse Anti-UCHL1/RBITC Conjugated antibody (bsm-51100M-RBITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bsm-51100M-RBITC
英文名稱1 Mouse Anti-UCHL1/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標(biāo)記的小鼠抗泛素硫酯酶L1單克隆抗體
別    名 Gracile axonal dystrophy; Neuron cytoplasmic protein 9.5; Park 5; Park5; Parkinson Disease 5; PGP 9.5; PGP9.5; PGP95; Protein gene product 9.5; Ubiquitin C terminal esterase L1; Ubiquitin C terminal hydrolase (neuron specific); Ubiquitin C terminal hydrolase; Ubiquitin carboxyl terminal esterase L1; Ubiquitin carboxyl terminal hydrolase isozyme L1; Ubiquitin carboxyl-terminal hydrolase isozyme L1; Ubiquitin thioesterase L1; Ubiquitin thiolesterase; Ubiquitin thiolesterase L1; UCH L1; UCH-L1; UCHL1; UCHL1_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號 3C2
交叉反應(yīng) Human, 
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 25kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 Recombinant human UCHL1
亞    型 IgG1
純化方法 affinity purified by Protein G
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
The protein encoded by this gene belongs to the peptidase C12 family. This enzyme is a thiol protease that hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. This gene is specifically expressed in the neurons and in cells of the diffuse neuroendocrine system. Mutations in this gene may be associated with Parkinson disease.[provided by RefSeq, Sep 2009]

Function:
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.

Subcellular Location:
Cytoplasm. Endoplasmic reticulum membrane. About 30% of total UCHL1 is associated with membranes in brain.

Tissue Specificity:
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.

Post-translational modifications:
O-glycosylated.

DISEASE:
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.

Similarity:
Belongs to the peptidase C12 family.

Database links:

Entrez Gene: 7345 Human

Entrez Gene: 22223 Mouse

Entrez Gene: 396637 Pig

Entrez Gene: 29545 Rat

Entrez Gene: 101117250 Sheep

Entrez Gene: 325119 Zebrafish

Omim: 191342 Human

SwissProt: P09936 Human

SwissProt: Q9R0P9 Mouse

SwissProt: Q6SEG5 Pig

SwissProt: Q00981 Rat

Unigene: 518731 Human

Unigene: 29807 Mouse

Unigene: 107213 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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