货号：MAB2162

品牌：Merck Millipore

规格：100UL

目录价：￥7049.00

市场价格：￥5991.65

会员价格：￥5639.20

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: 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. View All» Epitope: a.a. 495-648 View All» Immunogen: ALDP fragment from aa 495-648 as a fusion protein View All» Clone: 1AL-2B4 View All» Isotype: IgG1 View All» Species Reactivity: Human View All» Application Notes: 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. View All» Presentation: Ascites. Liquid, does not contain any preservative. View All» Storage Conditions: 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. View All» UniProt Number: P33897 View All» Entrez Gene Number: NM_000033.2 View All» Gene Symbol:

• ABCD1

• ALD

• AMN

• adrenoleukodystrophy/adrenomyeloneuropathy

• ALDP

• X-ALD

• ABC42

• adrenoleukodystrophy
  View All» Alternate Names: ALDP View All» 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. View All» Key Applications:
  • ELISA

  • Western Blotting

  • Immunocytochemistry

  • Immunohistochemistry
    View All» Entrez Gene Summary: 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. View All» UniProt Summary: 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. View All» Brand Family: Chemicon View All» Product Name: Anti-Adrenoleukodystrophy Protein Antibody, a.a. 495-648, clone 1AL-2B4 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Ascites View All» Host: Mouse View All»
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