{"product_id":"nsj-abcd1-antibody-atp-binding-cassette-sub-family-d-member-1-primary-antibody","title":"ABCD1 Antibody \/ ATP-binding cassette sub-family D member 1","description":"\u003cp\u003eABCD1 antibody detects ATP-binding cassette sub-family D member 1, a peroxisomal membrane transporter involved in the import of very-long-chain fatty acids (VLCFAs) into peroxisomes for degradation. ABCD1 belongs to the ABC transporter family and is encoded by a gene on the X chromosome. Loss-of-function mutations in ABCD1 cause X-linked adrenoleukodystrophy (X-ALD), a severe metabolic disorder characterized by accumulation of VLCFAs in plasma and tissues, leading to demyelination and adrenal insufficiency.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;Research using ABCD1 antibody has demonstrated its critical role in peroxisomal lipid metabolism. ABCD1 works as a half-transporter that dimerizes, either with itself or with other family members such as ABCD2, to import VLCFAs across the peroxisomal membrane. Disruption of this transport pathway leads to impaired beta-oxidation and pathological lipid accumulation. In the nervous system, VLCFA buildup damages myelin and causes neuroinflammation, while in endocrine tissues it impairs adrenal steroidogenesis.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;Clinical studies highlight ABCD1 as the causative gene in X-ALD, which manifests in childhood cerebral ALD, adrenomyeloneuropathy (AMN), and Addison-only disease. Mutations in ABCD1 result in variable phenotypes, even within the same family, suggesting additional genetic and environmental modifiers. Antibody-based detection of ABCD1 supports diagnostic studies and research into genotype-phenotype correlations.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;In therapeutic research, ABCD1 is a target for gene therapy and stem cell transplantation, both of which aim to restore peroxisomal function and prevent neurological decline. Monitoring ABCD1 expression with antibodies enables evaluation of therapeutic efficacy. Studies also indicate compensatory roles for ABCD2 and ABCD3 transporters, making antibody-based tools essential for dissecting overlapping functions.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;Antibodies against ABCD1 are validated for immunohistochemistry, western blot, and immunofluorescence. These reagents allow detection of peroxisomal localization, quantification of expression, and investigation of functional restoration in therapeutic models. By enabling precise measurement of ABCD1, they support basic, clinical, and translational research.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;NSJ Bioreagents supplies this ABCD1 antibody for studies in peroxisomal metabolism, neurodegeneration, and rare genetic disorders.\u003c\/p\u003e\u003ch3\u003eSpecifications\u003c\/h3\u003e\u003ctable style=\"width:100%; border-collapse:collapse;\"\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eFamily\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003ePrimary antibody\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eFormulation\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eRabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol, 0.4-0.5mg\/ml BSA\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eFormat\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eLiquid\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eClone\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003e30A99\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eHost Animal\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eRabbit\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eClonality\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eRecombinant Rabbit Monoclonal\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eIsotype\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eRabbit IgG\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eSpecies Reactivity\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eHuman, Mouse, Rat\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eApplication\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eWB, ICC, IF, FACS\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eApplication Details\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eWestern blot: 1:500-1:2000,Immunocytochemistry\/Immunofluorescence: 1:50-1:200,Flow Cytometry: 1:50\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eApplication Note\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eOptimal dilution of the ABCD1 antibody should be determined by the researcher.\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eImmunogen\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eA synthesized peptide derived from human ABCD1 \/ ALD was used as the immunogen for the ABCD1 antibody.\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eBuffer\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eRabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol, 0.4-0.5mg\/ml BSA.\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003ePurity\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eAffinity-chromatography\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eStorage\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eStore the ABCD1 antibody at -20oC.\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eLimitation\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eThis ABCD1 antibody is available for research use only.\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eUniprot #\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eP33897\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eStatus\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eAvailable\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003ePDF Link\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003e\u003ca href=\"http:\/\/www.nsjbio.com\/tds-pdf\/abcd1-antibody-atp-binding-cassette-sub-family-d-member-1-fy12269-nsj-bioreagents\" target=\"_blank\" rel=\"noopener noreferrer\"\u003ehttp:\/\/www.nsjbio.com\/tds-pdf\/abcd1-antibody-atp-binding-cassette-sub-family-d-member-1-fy12269-nsj-bioreagents\u003c\/a\u003e\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eTitle\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eABCD1 Antibody \/ ATP-binding cassette sub-family D member 1\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left; vertical-align:top; padding:6px; border-bottom:1px solid #eee;\"\u003eDescription\u003c\/th\u003e\n\u003ctd style=\"padding:6px; border-bottom:1px solid #eee;\"\u003eABCD1 antibody detects ATP-binding cassette sub-family D member 1, a peroxisomal membrane transporter involved in the import of very-long-chain fatty acids (VLCFAs) into peroxisomes for degradation. ABCD1 belongs to the ABC transporter family and is encoded by a gene on the X chromosome. Loss-of-function mutations in ABCD1 cause X-linked adrenoleukodystrophy (X-ALD), a severe metabolic disorder characterized by accumulation of VLCFAs in plasma and tissues, leading to demyelination and adrenal insufficiency.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;Research using ABCD1 antibody has demonstrated its critical role in peroxisomal lipid metabolism. ABCD1 works as a half-transporter that dimerizes, either with itself or with other family members such as ABCD2, to import VLCFAs across the peroxisomal membrane. Disruption of this transport pathway leads to impaired beta-oxidation and pathological lipid accumulation. In the nervous system, VLCFA buildup damages myelin and causes neuroinflammation, while in endocrine tissues it impairs adrenal steroidogenesis.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;Clinical studies highlight ABCD1 as the causative gene in X-ALD, which manifests in childhood cerebral ALD, adrenomyeloneuropathy (AMN), and Addison-only disease. Mutations in ABCD1 result in variable phenotypes, even within the same family, suggesting additional genetic and environmental modifiers. Antibody-based detection of ABCD1 supports diagnostic studies and research into genotype-phenotype correlations.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;In therapeutic research, ABCD1 is a target for gene therapy and stem cell transplantation, both of which aim to restore peroxisomal function and prevent neurological decline. Monitoring ABCD1 expression with antibodies enables evaluation of therapeutic efficacy. Studies also indicate compensatory roles for ABCD2 and ABCD3 transporters, making antibody-based tools essential for dissecting overlapping functions.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;Antibodies against ABCD1 are validated for immunohistochemistry, western blot, and immunofluorescence. These reagents allow detection of peroxisomal localization, quantification of expression, and investigation of functional restoration in therapeutic models. By enabling precise measurement of ABCD1, they support basic, clinical, and translational research.\u0026lt;br\u0026gt;\u0026lt;br\u0026gt;NSJ Bioreagents supplies this ABCD1 antibody for studies in peroxisomal metabolism, neurodegeneration, and rare genetic disorders.\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/table\u003e","brand":"NSJ Bioreagents","offers":[{"title":"100 ul","offer_id":51543737434392,"sku":"FY12269","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/get_image_4fff948f-c048-4e2e-afa6-eb13da19d15b.jpg?v=1768135005","url":"https:\/\/danabiosci.com\/products\/nsj-abcd1-antibody-atp-binding-cassette-sub-family-d-member-1-primary-antibody","provider":"Dana Bioscience","version":"1.0","type":"link"}