ATP Binding Cassette Transporter D1 (ABCD1) Antibody

260€ (100 µl)
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935106861
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name
ATP Binding Cassette Transporter D1 (ABCD1) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx130575
tested applications
WB, IHC, IF/ICC
Description
ABCD1 Antibody is a Rabbit Polyclonal against ABCD1.
Documents del producto
Instrucciones
Data sheet
Product specifications
Category | Primary Antibodies |
Immunogen Target | ATP Binding Cassette Transporter D1 (ABCD1) |
Host | Rabbit |
Reactivity | Human |
Recommended Dilution | 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. |
Clonality | Polyclonal |
Conjugation | Unconjugated |
Purification | Purified by antigen-specific affinity chromatography, followed by Protein A affinity chromatography. |
Size 1 | 100 µl |
Size 2 | 200 µl |
Size 3 | 1 ml |
Form | Liquid |
Tested Applications | WB, IHC, IF/ICC |
Buffer | 0.01 M PBS, pH 7.4, containing 0.05% Proclin-300, 50% glycerol. |
Availability | Shipped within 5-7 working days. |
Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
Dry Ice | No |
UniProt ID | P33897 |
Alias | ABC42,ALD,ALDP,AMN,Adrenoleukodystrophy protein |
Background | Antibody anti-ABCD1 |
Status | 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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