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自水解酶結構域5蛋白抗體廠家

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更新時間:2017-03-03 01:58:53瀏覽次數:385次

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英文名稱 ABHD5
中文名稱 自水解酶結構域5蛋白抗體
別    名 1-acylglycerol-3-phosphate O-acyltransferase ABHD5; ABHD5; ABHD5_HUMAN; Abhydrolase domain containing 5; Abhydrolase domain containing protein 5; Abhydrolase domain-containing protein 5; CDS; CGI 58; CGI58; CGI58 protein; IECN2; Lipid droplet-binding protein CGI-58; MGC8731; NCIE2.
自水解酶結構域5蛋白抗體廠家
研究領域 腫瘤  心血管  細胞生物  免疫學  信號轉導  脂蛋白  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat, Dog, Cow, Rabbit, Sheep, 
產品應用 WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蠟切片需做抗原修復)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 39kDa
細胞定位 細胞漿 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ADHD5
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide
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.
自水解酶結構域5蛋白抗體廠家
產品介紹 background:
Abhd5 belongs to a large family of proteins defined by an alpha/beta hydrolase fold, and contains three sequence motifs that correspond to a catalytic triad found in the esterase/lipase/thioesterase subfamily. It differs from other members of this subfamily in that its putative catalytic triad contains an asparagine instead of the serine residue. Mutations in this gene have been associated with Chanarin-Dorfman syndrome, a triglyceride storage disease with impaired long-chain fatty acid oxidation. Widely expressed in various tissues, including skin, lymphocytes, liver, skeletal muscle and brain.

Function:
Lysophosphatidic acid acyltransferase which functions in phosphatidic acid biosynthesis. May regulate the cellular storage of triacylglycerol through activation of the phospholipase PNPLA2. Involved in keratinocyte differentiation.

Subunit:
Interacts with ADRP, PLIN and PNPLA2 (By similarity).

Subcellular Location:
Cytoplasm. Lipid droplet.

Tissue Specificity:
Widely expressed in various tissues, including lymphocytes, liver, skeletal muscle and brain. Expressed by upper epidermal layers and dermal fibroblasts in skin, hepatocytes and neurons.

DISEASE:
Defects in ABHD5 are the cause of Chanarin-Dorfman syndrome (CDS) [MIM:275630]; also called triglyceride storage disease with impaired long-chain fatty acid oxidation or neutral lipid storage disease with ichthyosis. CDS is an autosomal recessive inborn error of lipid metabolism with multisystemic accumulation of triglycerides although plasma concentrations are normal. Clinical characteristics are congenital generalized ichthyosis, vacuolated leukocytes, hepatomegaly, myopathy, cataracts, neurosensory hearing loss and developmental delay. The disorder presents at birth with generalized, fine, white scaling of the skin and a variable degree of erythema resembling non-bullous congenital ichthyosiform erythroderma.

Similarity:
Belongs to the peptidase S33 family. ABHD4/ABHD5 subfamily.

Database links:
UniProtKB/Swiss-Prot: Q8WTS1.1

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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