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        ABHD5 Rabbit pAb (bs-5028R)  
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        產品編號 bs-5028R
        英文名稱 ABHD5 Rabbit pAb
        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.  
        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
        克 隆 號
        交叉反應 Rat (predicted: Human,Mouse,Rabbit,Sheep,Cow,Dog)
        產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
        not yet tested in other applications.
        optimal dilutions/concentrations should be determined by the end user.
        理論分子量 39 kDa
        檢測分子量
        細胞定位 細胞漿 
        性    狀 Liquid
        濃    度 1mg/ml
        免 疫 原 KLH conjugated synthetic peptide derived from human ADHD5: 281-349/349 
        亞    型 IgG
        純化方法 affinity purified by Protein A
        緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
        保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
        注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
        PubMed PubMed
        產品介紹 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.

        SWISS:
        Q8WTS1

        Gene ID:
        51099

        Database links:

        Entrez Gene: 51099 Human

        Entrez Gene: 67469 Mouse

        Entrez Gene: 497624 Pig

        Entrez Gene: 316122 Rat

        Entrez Gene: 100125355 Sheep

        Omim: 604780 Human

        SwissProt: Q8WTS1 Human

        SwissProt: Q9DBL9 Mouse

        SwissProt: Q5EE05 Pig

        SwissProt: Q6QA69 Rat

        Unigene: 19385 Human

        Unigene: 280254 Mouse

        Unigene: 12459 Rat



        產品圖片
        Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-ABHD5 Polyclonal Antibody, Unconjugated(bs-5028R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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