ATP Binding Cassette Transporter D1 (ABCD1) Antibody

Product specifications

Primary Antibodies

ATP Binding Cassette Transporter D1 (ABCD1)

Rabbit

Human

WB: 0.01-2 µg/ml, IHC: 5-20 µg/ml, IF/ICC: 5-20 µg/ml. Optimal dilutions/concentrations should be determined by the end user.

Polyclonal

Unconjugated

Purified by antigen-specific affinity chromatography, followed by Protein A affinity chromatography.

100 µl

200 µl

1 ml

Liquid

WB, IHC, IF/ICC

0.01 M PBS, pH 7.4, containing 0.05% Proclin-300, 50% glycerol.

Shipped within 5-7 working days.

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.

No

P33897

ABC42,ALD,ALDP,AMN,Adrenoleukodystrophy protein

Antibody anti-ABCD1

RUO

Descripción

ATP Binding Cassette Transporter D1 (ABCD1), part of the peroxisomal ABC transporter family, facilitates the import of very-long-chain fatty acids (VLCFAs) into peroxisomes for degradation. Mutations in the ABCD1 gene cause X-linked adrenoleukodystrophy (X-ALD), a neurodegenerative disorder characterized by the accumulation of VLCFAs in the central nervous system, adrenal glands, and plasma. ABCD1 dysfunction leads to demyelination, adrenal insufficiency, and progressive neurological decline. The protein is ubiquitously expressed, with high levels in tissues involved in lipid metabolism, including the liver, adrenal glands, and brain. Therapies targeting ABCD1, such as gene therapy and dietary interventions to reduce VLCFA levels, have shown promise in mitigating disease progression. Its essential role in lipid homeostasis highlights its broader relevance in metabolic and peroxisomal disorders.

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