Human ATP Binding Cassette Transporter D1 (ABCD1) Protein

247€ (10 µg)
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935106861
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name
Human ATP Binding Cassette Transporter D1 (ABCD1) Protein
category
Proteins and Peptides
provider
Abbexa
reference
abx168633
tested applications
WB, SDS-PAGE
Description
Human ABCD1 Protein is a recombinant Human protein produced in a Prokaryotic expression system (E. coli).
Documents del producto
Instrucciones
Data sheet
Product specifications
Category | Proteins and Peptides |
Immunogen Target | ATP Binding Cassette Transporter D1 (ABCD1) |
Host | E. coli |
Origin | Human |
Conjugation | Unconjugated |
Observed MW | Molecular Weight: Calculated MW: 62.1 kDa Concentration: Prior to lyophilization: 200 µg/ml Sequence Fragment: Val102-Thr386 Tag: N-terminal His tag and GST tag |
Expression | Recombinant |
Purity | > 90% |
Size 1 | 10 µg |
Size 2 | 50 µg |
Size 3 | 100 µg |
Size 4 | 200 µg |
Size 5 | 500 µg |
Form | Lyophilized To keep the original salt concentration, we recommend reconstituting to the original concentration prior to lyophilization (see Concentration) in ddH2O. If a lower concentration is required, dilute in PBS, pH 7.4. If a higher concentration is required, the product can be reconstituted directly in PBS, pH 7.4, though please note that this will change the overall salt concentration. The stock concentration should be between 0.1-1.0 mg/ml. Do not vortex. |
Tested Applications | WB, SDS-PAGE |
Buffer | Prior to lyophilization: PBS, pH 7.4, containing 0.01% Sarcosyl, 1 mM DTT, 5% Trehalose and Proclin-300. |
Availability | Shipped within 5-7 working days. |
Storage | Store at 2-8 °C for up to one month. Store at -80 °C for up to one year. Avoid repeated freeze/thaw cycles. |
Dry Ice | No |
UniProt ID | P33897 |
Alias | ABC42,ALD,ALDP,AMN,Adrenoleukodystrophy protein |
Background | Protein ABCD1 |
Status | RUO |
Note | This product is for research use only. Not for human consumption, cosmetic, therapeutic or diagnostic use. |
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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