产品基本信息

• 产品货号 BD-PT0049
• 别名 ABCD1; ALD; ATP-binding cassette sub-family D member 1; Adrenoleukodystrophy protein; ALDP
• 产品名称 ABCD1 Rabbit Polyclonal Antibody
• 类别 抗体产品
• 基因名称 ABCD1
• 蛋白名称 ATP-binding cassette sub-family D member 1
• Clonality Polyclonal
• 推荐应用 WB,ELISA
• 反应种属 Human,Rat,Mouse
• 浓度 1 mg/ml
• 存储缓冲液 Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
• Human Gene ID 215
• Human Gene Link http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=215
• Human Swissprot No. P33897
• Human Swissprot Link http://www.uniprot.org/uniprotkb/P33897/entry
• Mouse Swissprot No. P48410
• Mouse Swissprot Link http://www.uniprot.org/uniprot/P48410
• 免疫原 The antiserum was produced against synthesized peptide derived from human ABCD1. AA range:531-580
• 特异性 ABCD1 Polyclonal Antibody detects endogenous levels of ABCD1 protein.
• 稀释度 WB 1:500-1:2000, ELISA 1:40000.Not yet tested in other applications.
• 预测分子量 75kDa
• 运输及保存条件 Biological ice bag transportation. Store at -20°C for at least 12 months(Do not lower than -25°C). Avoid freeze/thaw cycles
• 宿主 Rabbit
• 同种型 Rabbit,IgG
• 背景介绍 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. This peroxisomal membrane protein is likely involved in the peroxisomal transport or catabolism of very long chain fatty acids. Defects in this gene have been identified as the underlying cause of adrenoleukodystrophy, an X-chromosome recessively inherited demyelinating disord
• 组织表达 Brain,Pancreas,
• 细胞定位 Peroxisome membrane ; Multi-pass membrane protein . Mitochondrion membrane ; Multi-pass membrane protein. Lysosome membrane ; Multi-pass membrane protein. Endoplasmic reticulum membrane ; Multi-pass membrane protein.
• 信号通路 ABC transporters;
• 功能 disease:Defects in ABCD1 are the cause of adrenoleukodystrophy X-linked (X-ALD) [MIM:300100]. X-ALD is a peroxisomal metabolic disorder characterized by progressive multifocal demyelination of the central nervous system and by peripheral adrenal insufficiency (Addison disease). It results in mental deterioration, corticospinal tract dysfunction, and cortical blindness. Different clinical manifestations exist like: cerebral childhood ALD (CALD), adult cerebral ALD (ACALD), adrenomyeloneuropathy (AMN) and "Addison disease only" (ADO) phenotype.,disease:Microdeletions in ABCD1 are involved in the contiguous ABCD1/DXS1375E deletion syndrome (CADDS) [MIM:300475]. Patients manifest profound neonatal hypotonia, subsequent failure to thrive, and cholestatic liver disease.,function:Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.,similarity:Belongs to the ABC transporter family. ALD subfamily.,similarity:Contains 1 ABC transmembrane type-1 domain.,similarity:Contains 1 ABC transporter domain.,subunit:Can form homo- and heterodimers with ABCD2/ALDR and ABCD3/PMP70. Dimerization is necessary to form an active transporter. Interacts with PEX19.,
• 纯化 The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

图片

	Western blot analysis of lysates from Jurkat cells, using ABCD1 Antibody. The lane on the right is blocked with the synthesized peptide.
	Western blot analysis of the lysates from HeLa cells using ABCD1 antibody.
	Western Blot analysis of Hela cells using ABCD1 Polyclonal Antibody

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