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        Rabbit Anti-ABCD2/BF647 Conjugated antibody (bs-10646R-BF647)
        訂購熱線:400-901-9800
        訂購郵箱:sales@bioss.com.cn
        訂購QQ:  400-901-9800
        技術支持:techsupport@bioss.com.cn
        說 明 書: 100ul  
        100ul/2980.00元
        大包裝/詢價
        產品編號 bs-10646R-BF647
        英文名稱 Rabbit Anti-ABCD2/BF647 Conjugated antibody
        中文名稱 BF647標記的三磷酸腺苷結合盒轉運蛋白2抗體
        別    名 ABC39; Abcd2; ABCD2_HUMAN; Adrenoleukodystrophy-like 1; Adrenoleukodystrophy-related protein; ALDL1; ALDR; ALDRP; ATP-binding cassette sub-family D member 2; hALDR.  
        規格價格 100ul/2980元 購買        大包裝/詢價
        說 明 書 100ul  
        研究領域 腫瘤  心血管  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 Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Horse, 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 83kDa
        性    狀 Lyophilized or Liquid
        濃    度 1mg/ml
        免 疫 原 KLH conjugated synthetic peptide derived from human ABCD2
        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 protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ALD subfamily, which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. The function of this peroxisomal membrane protein is unknown; however this protein is speculated to function as a dimerization partner of ABCD1 and/or other peroxisomal ABC transporters. Mutations in this gene have been observed in patients with adrenoleukodystrophy, a severe demyelinating disease. This gene has been identified as a candidate for a modifier gene, accounting for the extreme variation among adrenoleukodystrophy phenotypes. This gene is also a candidate for a complement group of Zellweger syndrome, a genetically heterogeneous disorder of peroxisomal biogenesis. [provided by RefSeq, Jul 2008]

        Function:
        Probable transporter.

        Subunit:
        Can form heterodimers with ABCD1/ALD and ABCD3/PMP70. Dimerization is necessary to form an active transporter. Interacts with PEX19.

        Subcellular Location:
        Peroxisome membrane; Multi-pass membrane protein.

        Tissue Specificity:
        Predominantly expressed in brain and heart.

        Similarity:
        Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily.
        Contains 1 ABC transmembrane type-1 domain.
        Contains 1 ABC transporter domain.

        Database links:

        Entrez Gene: 225 Human

        Entrez Gene: 26874 Mouse

        Entrez Gene: 84356 Rat

        Omim: 601081 Human

        SwissProt: Q9UBJ2 Human

        SwissProt: Q61285 Mouse

        SwissProt: Q9QY44 Rat

        Unigene: 117852 Human

        Unigene: 295456 Mouse

        Unigene: 19678 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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