ABCD1 antibody

Product specifications

Primary Antibodies

ATP-binding cassette, sub-family D(ALD), member 1 (ABCD1)

Mouse

Human

WB: 1:200-1:1000; IF: 1:20-1:200

monoclonal

Unconjugated

IgG1

9D11

80 kDa

≥95% as determined by SDS-PAGE

Protein A+G purification

100µg

liquid

ELISA, WB

PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20℃ for 12 months(Avoid repeated freeze / thaw cycles.)

P33897

215

ABC42,ALD,ALDP,AMN,Adrenoleukodystrophy protein

Antibody anti-ABCD1

RUO

Mol. Weight 80 kDa

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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