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MAB2164 Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6

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MAB2164
100 µL  
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      Descripción

      Replacement Information

      Ofertas especiales

      Tabla espec. clave

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HELISA, WB, ICC, IHCMAscitesMonoclonal Antibody
      Description
      Catalogue NumberMAB2164
      Brand Family Chemicon®
      Trade Name
      • Chemicon
      DescriptionAnti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6
      Alternate Names
      • ALDP
      References
      Product Information
      FormatAscites
      PresentationAscites. Liquid, does not contain any preservative.
      Quality LevelMQ100
      Applications
      ApplicationDetect Adrenoleukodystrophy Protein using this Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 validated for use in ELISA, WB, IC, IH.
      Key Applications
      • ELISA
      • Western Blotting
      • Immunocytochemistry
      • Immunohistochemistry
      Application NotesELISA: 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.
      Biological Information
      ImmunogenALDP1 fragment from aa 279 to 482 as a fusion protein
      Epitopea.a. 279-482
      Clone2AL-1D6
      HostMouse
      SpecificityHuman 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.
      IsotypeIgG1
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThe 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.
      Gene Symbol
      • ABCD1
      • ALD
      • AMN
      • adrenoleukodystrophy/adrenomyeloneuropathy
      • ALDP
      • X-ALD
      • ABC42
      • adrenoleukodystrophy
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Usage Statement
      • 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.
      Storage and Shipping Information
      Storage ConditionsMaintain at -20°C in undiluted aliquots up to 12 months. Avoid repeated freeze/thaw cycles.
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Número de referencia GTIN
      MAB2164 04053252578366

      Documentation

      Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 Ficha datos de seguridad (MSDS)

      Título

      Ficha técnica de seguridad del material (MSDS) 

      Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 Certificados de análisis

      CargoNúmero de lote
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 2558431 2558431
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3162486 3162486
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3229403 3229403
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3317083 3317083
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3502189 3502189
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3506673 3506673
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3597334 3597334
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3720132 3720132
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3743889 3743889
      MOUSE ANTI-ADRENOLEUKODYSTROPHY PROTEIN (ALDP) - 3793751 3793751

      Referencias bibliográficas

      Visión general referenciasPub Med ID
      Psychosine-induced alterations in peroxisomes of twitcher mouse liver.
      Miguel Agustin Contreras,Ehtishamul Haq,Takuhiro Uto,Inderjit Singh,Avtar Kaur Singh
      Archives of biochemistry and biophysics  477  2008

      Mostrar resumen Artículo Texto completo
      18602885 18602885
      Topology of ATP-binding domain of adrenoleukodystrophy gene product in peroxisomes.
      Contreras, M, et al.
      Arch. Biochem. Biophys., 334: 369-79 (1996)  1996

      Mostrar resumen
      8900413 8900413
      A close relative of the adrenoleukodystrophy (ALD) gene codes for a peroxisomal protein with a specific expression pattern.
      Lombard-Platet, G, et al.
      Proc. Natl. Acad. Sci. U.S.A., 93: 1265-9 (1996)  1996

      Mostrar resumen
      8577752 8577752
      X-linked adrenoleukodystrophy.
      Aubourg, P and Mandel, J L
      Ann. N. Y. Acad. Sci., 804: 461-76 (1996)  1996

      8993565 8993565
      The gene responsible for adrenoleukodystrophy encodes a peroxisomal membrane protein.
      Mosser, J, et al.
      Hum. Mol. Genet., 3: 265-71 (1994)  1993

      Mostrar resumen
      8004093 8004093

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      Categorías

      Life Science Research > Antibodies and Assays > Primary Antibodies