merck millipore,默克密理博,MAB2162,Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4

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merck millipore,默克密理博,MAB2162,Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4
产品名称：Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4
产品型号：MAB2162
Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4 detects level of Adrenoleukodystrophy Protein & has been published & validated for use in ELISA, WB, IC, IH.

merck millipore,默克密理博,MAB2162,Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4

产品介绍

merck millipore,默克密理博,MAB2162,Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4

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	MAB2162
品牌系列	Chemicon®
商名	Chemicon
描述	Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4
Alternate Names	ALDP

产品信息
格式	Ascites
演示	Ascites. Liquid, does not contain any preservative.

应用
应用	Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4 detects level of Adrenoleukodystrophy Protein & has been published & 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 Western blot: 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	ALDP fragment from aa 495-648 as a fusion protein
表位	a.a. 495-648
克隆	1AL-2B4
宿主	Mouse
特异性	Human adrenoleukodystrophy protein (ALDP). No cross-reactivity on mouse ALDP, mouse ALDRP and PMP 70. MAB2162 can be used in Western blot, immunofluorescence or immunohistochemical studies to analyze the ALD 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 mosaicism 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	ABCD1 ALD AMN adrenoleukodystrophy/adrenomyeloneuropathy ALDP X-ALD ABC42 adrenoleukodystrophy
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 after date of receipt. Avoid repeated freeze/thaw cycles. Important Note: During shipment, small volumes of product will occasionally become entrapped in the seal of the product vial. For products with volumes of 200 μl or less, we recommend gently tapping the vial on a hard surface or briefly centrifuging the vial in a tabletop centrifuge to dislodge any liquid in the container's cap.

储存和货运信息

存储条件

Maintain at -20°C in undiluted aliquots up to 12 months after date of receipt. Avoid repeated freeze/thaw cycles.

Important Note: During shipment, small volumes of product will occasionally become entrapped in the seal of the product vial. For products with volumes of 200 μl or less, we recommend gently tapping the vial on a hard surface or briefly centrifuging the vial in a tabletop centrifuge to dislodge any liquid in the container's cap.