Rekombinanter ABCD1 Antikörper

Produktnummer ABIN7880023

Der Kaninchen Monoklonal anti-ABCD1 Antikörper (Klon 30A99) (ABIN7880023) detektiert spezifisch ABCD1 in WB, FACS, ICC und IF. Dieser Antikörper reagiert spezifisch mit Proben aus Human, Maus und Ratte.

Kurzübersicht für Rekombinanter ABCD1 Antikörper (ABIN7880023)

Target: ABCD1 (ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1))

Antikörpertyp: Recombinant Antibody

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Monoklonal

Konjugat: Dieser ABCD1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Flow Cytometry (FACS), Immunocytochemistry (ICC), Immunofluorescence (IF)

Klon: 30A99

Produktdetails anti-ABCD1 Antikörper

Verwendungszweck: ABCD1 Antibody / ATP-binding cassette sub-family D member 1

Aufreinigung: Affinity-chromatography

Immunogen: A synthesized peptide derived from human ABCD1 / ALD was used as the immunogen for the ABCD1 antibody.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the ABCD1 antibody should be determined by the researcher.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Rabbit IgG in phosphate buffered saline, pH 7.4, 150 mM NaCl, 0.02 % sodium azide and 50 % glycerol, 0.4-0.5 mg/mL BSA

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: -20 °C

Informationen zur Lagerung: Store the ABCD1 antibody at -20oC.

Details zu ABCD1

Target: ABCD1 (ATP-Binding Cassette, Sub-Family D (Ald), Member 1 (ABCD1))

Andere Bezeichnung: ABCD1

Hintergrund: ABCD1 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.

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.

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.

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.

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.

NSJ Bioreagents supplies this ABCD1 antibody for studies in peroxisomal metabolism, neurodegeneration, and rare genetic disorders.

UniProt: P33897

Pathways: Monocarboxylic Acid Catabolic Process