Human ATP Binding Cassette Transporter D1 (ABCD1) Protein

Este producto es parte de ABCD - ATP Binding Cassette Transporter D
Product Graph
247€ (10 µg)

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935106861
info@markelab.com
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
Descargar

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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