- 当前位置：首页 > >Merck Millipore > 生命科学研究
- 生命科学研究
-
-
merck millipore,默克密理博,MAB2164,Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6
产品名称：Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6
产品型号：MAB2164
Detect Adrenoleukodystrophy Protein using this Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 validated for use in ELISA, WB, IC, IH.
merck millipore,默克密理博,MAB2164,Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6
- 产品介绍
- merck millipore,默克密理博,MAB2164,Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H IHC, ELISA, WB, ICC M Ascites Monoclonal Antibody 描述 产品目录编号 MAB2164 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 Alternate Names - ALDP
产品信息 格式 Ascites 演示 Ascites. Liquid, does not contain any preservative. 应用 应用 Detect Adrenoleukodystrophy Protein using this Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 validated for use in ELISA, WB, IC, IH. 主要应用 - Immunohistochemistry
- ELISA
- Western Blotting
- Immunocytochemistry
应用说明 ELISA: 1:500-1:5,000
Immunoblotting: 1:500-1:5,000
Immunohistochemistry: 1:500-1:5,000
Immunocytochemistry: 1:500-1:5,000
Optimal working dilutions must be determined by the end user.生物信息 免疫原品种 ALDP1 fragment from aa 279 to 482 as a fusion protein 表位 a.a. 279-482 克隆 2AL-1D6 宿主 Mouse 特异性 Human ALDP. No cross reactivity with mouse ALDRP, PMP70. Cross reactivity to an unidentified 80 kD MW protein is present in lymphoblastoid cells, but not in several other cells or tissues tested. MAB2164 can be used to analyze ALDP protein in cells and tissues from normal individuals, or from patients with adrenoleukodystrophy (ALD) or Zellweger syndrome and related peroxisomal diseases, and provides an excellent marker for peroxisomes (Mosser et al. 1994). About 70% of ALD male patients have no detectable ALDP in fibroblasts or leukocytes. A similar proportion of carrier females may show mosaïcism for the presence or absence of ALDP. 同种型 IgG1 品种反应性 Human 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_000033.2
Entrez基因汇总 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 disorder of the nervous system. 基因符号 - adrenoleukodystrophy
- ABCD1
- ALD
- AMN
- adrenoleukodystrophy/adrenomyeloneuropathy
- ALDP
- X-ALD
- ABC42
UniProt编号 - P33897
UniProt汇总 FUNCTION: SwissProt: P33897 # Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.
SIZE: 745 amino acids; 82937 Da
SUBUNIT: Can form homo- and heterodimers with ABCD2/ALDR and ABCD3/PMP70. Dimerization is necessary to form an active transporter. Interacts with PEX19.
SUBCELLULAR LOCATION: Peroxisome membrane; Multi-pass membrane protein.
DISEASE: SwissProt: P33897 # Defects in ABCD1 are the cause of X-linked adrenoleukodystrophy (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. & 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.
SIMILARITY: SwissProt: P33897 ## Belongs to the ABC transporter family. ALD subfamily. & Contains 1 ABC transmembrane type-1 domain. & Contains 1 ABC transporter domain.产品使用声明 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain at -20°C in undiluted aliquots up to 12 months. Avoid repeated freeze/thaw cycles. 包装信息 数量 100 µL
上一件merck millipore产品：merck millipore,默克密理博,ABN271,Anti-TARDBP Antibody
下一件merck millipore产品：merck millipore,默克密理博,481413,Aurora A Kinase, His•Tag, Human, Recombinant, S. frugiperda
相关产品
merck millipore,默克密理博,524751,Phosphodiesterase 7A1, Human, Recombinant, S. frugiperda
merck millipore,默克密理博,124015,InSolution™ Akt Inhibitor IV - Calbiochem
merck millipore,默克密理博,SF-777,MAP1LC3B; Human, Cyanine 5, RNA Detection Probe,SmartFlare
merck millipore,默克密理博,ABN271,Anti-TARDBP Antibody
merck millipore,默克密理博,481413,Aurora A Kinase, His•Tag, Human, Recombinant, S. frugiperda
merck millipore,默克密理博,539630,Protein Phosphatase Inhibitor Set II - Calbiochem
merck millipore,默克密理博,ABS223,Anti-RAS-Related protein Ral-A Antibody
