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Rabbit Anti-Aminoacylase 1/BF594 Conjugated antibody (bs-6019R-BF594)
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訂購(gòu)QQ:  400-901-9800
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-6019R-BF594
英文名稱 Rabbit Anti-Aminoacylase 1/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的氨基?;?抗體
別    名 ACY 1; ACY1; ACY1D; ACYLASE; EC 3.5.1.14; N acyl L amino acid amidohydrolase; ACY1_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 免疫學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  通道蛋白  新陳代謝  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng)
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 46kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACY1/Aminoacylase 1
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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:
Aminoacylase 1 is a cytosolic, homodimeric, zinc binding enzyme that catalyzes the hydrolysis of acylated L amino acids to L amino acids and acyl group, and has been postulated to function in the catabolism and salvage of acylated amino acids. ACY1 has been assigned to chromosome 3p21.1, a region reduced to homozygosity in small cell lung cancer (SCLC), and its expression has been reported to be reduced or undetectable in SCLC cell lines and tumors. The amino acid sequence of human aminoacylase 1 is highly homologous to the porcine counterpart, and ACY1 is the first member of a new family of zinc binding enzymes.

Function:
Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate).

Subunit:
Homodimer. Interacts with SPHK1.

Subcellular Location:
Cytoplasm.

Tissue Specificity:
Expression is highest in kidney, strong in brain and weaker in placenta and spleen.

DISEASE:
Defects in ACY1 are the cause of aminoacylase-1 deficiency (ACY1D) [MIM:609924]. ACY1D results in a metabolic disorder manifesting with encephalopathy, unspecific psychomotor delay, psychomotor delay with atrophy of the vermis and syringomyelia, marked muscular hypotonia or normal clinical features. Epileptic seizures are a frequent feature. All affected individuals exhibit markedly increased urinary excretion of several N-acetylated amino acids.

Similarity:
Belongs to the peptidase M20A family.

Database links:
 

 

Entrez Gene: 768058 Cow

Entrez Gene: 95 Human

Entrez Gene: 109652 Mouse

Entrez Gene: 396930 Pig

Entrez Gene: 300981 Rat

Omim: 104620 Human

SwissProt: Q03154 Human

SwissProt: Q99JW2 Mouse

SwissProt: P37111 Pig

SwissProt: Q6AYS7 Rat

Unigene: 334707 Human

Unigene: 7165 Mouse

Unigene: 3679 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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