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Rabbit Anti-UCHL1+3/BF488 Conjugated antibody (bs-11677R-BF488)
訂購熱線:400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11677R-BF488
英文名稱 Rabbit Anti-UCHL1+3/BF488 Conjugated antibody
中文名稱 BF488標記的泛素硫酯酶L1+3抗體
別    名 Epididymis luminal protein 117; Epididymis secretory protein Li 53; HEL 117; HEL S 53; NDGOA; Neuron cytoplasmic protein 9.5; OTTHUMP00000218137; OTTHUMP00000218139; OTTHUMP00000218140; OTTHUMP00000218141; Park 5; PARK5; PGP 9.5; PGP9.5; PGP95; Protein gene product 9.5; Ubiquitin C terminal esterase L1; Ubiquitin C terminal hydrolase; Ubiquitin C terminal hydrolase L1; 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; UCHL1; UCHL1_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經生物學  信號轉導  泛素  Alzheimer's  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, Sheep, )
產品應用 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 KLH conjugated synthetic peptide derived from human UCHL1+3 (141-223aa)
亞    型 IgG
純化方法 affinity purified by Protein A
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.
產品介紹 background:
The two ubiquitin C-terminal hydrolase (UCH) enzymes, UCHL1 and UCHL3, deubiquitinate ubiquitin-protein conjugates and control the cellular balance of ubiquitin. UCHL1 and UCHL3 are both small proteins of ~220 amino acids that share more than 40% amino acid sequence identity. UCHL3 is universally expressed in all tissues, while UCHL1 is expressed exclusively in neuronal tissue, testis and ovary. The activity of UCHL3 is more than 200 fold higher than UCHL1 when a fluorogenic ubiquitin substrate is used. UCHL1 associates with monoubiquitin and UCHL3 binds to Nedd8, ubiquitin-like protein. UCHL1 and UCHL3 play a role in the regulation of neuronal development and spermatogenesis. UCHL1 is involved in the pathogenesis of Parkinson’s disease (PD) and Alzheimer’s disease (AD).

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.

Subunit:
Monomer. Homodimer. Interacts with SNCA (By similarity). Interacts with COPS5.

Subcellular Location:
Cytoplasm. Endoplasmic reticulum membrane; Lipid-anchor. Note=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

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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