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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | ELISA, WB, ICC, IHC | M | Ascites | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MAB2164 |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Anti-Adrenoleukodystrophy Protein Antibody, a.a. 279-482, clone 2AL-1D6 |
| Alternate Names |
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| Product Information | |
|---|---|
| Format | Ascites |
| Presentation | Ascites. Liquid, does not contain any preservative. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Detect 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 |
|
| Application Notes | 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. |
| Biological Information | |
|---|---|
| Immunogen | ALDP1 fragment from aa 279 to 482 as a fusion protein |
| Epitope | a.a. 279-482 |
| Clone | 2AL-1D6 |
| Host | Mouse |
| Specificity | 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. |
| Isotype | IgG1 |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| 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. |
| Gene Symbol |
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| UniProt Number |
|
| 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. |
| Product Usage Statements | |
|---|---|
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain at -20°C in undiluted aliquots up to 12 months. Avoid repeated freeze/thaw cycles. |
| Packaging Information | |
|---|---|
| Material Size | 100 µL |