ATP Binding Cassette Subfamily D Member 1 (ABCD1) Antibody

Este producto es parte de ABCD - ATP Binding Cassette Transporter D
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364€ (100 µg)

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935106861
info@markelab.com
name
ATP Binding Cassette Subfamily D Member 1 (ABCD1) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx230037
tested applications
ELISA, WB

Description

ABCD1 Antibody is a Mouse Monoclonal against ABCD1.

Documents del producto

Instrucciones
Data sheet
Descargar

Product specifications

Category
Primary Antibodies
Immunogen Target
ATP Binding Cassette Subfamily D Member 1 (ABCD1)
Host
Mouse
Reactivity
Human
Recommended Dilution
WB: 1/200 - 1/1000, IF/ICC: 1/20 - 1/200. Optimal dilutions/concentrations should be determined by the end user.
Clonality
Monoclonal
Conjugation
Unconjugated
Isotype
IgG1
Purity
≥ 95% (SDS-PAGE)
Purification
Purified by Protein A and Protein G affinity chromatography.
Size 1
100 µg
Form
Liquid
Tested Applications
ELISA, WB
Buffer
PBS, pH 7.3, with 0.02% sodium azide and 50% glycerol.
Availability
Shipped within 5-12 working days.
Storage
Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
Dry Ice
No
UniProt ID
P33897
Gene ID
215
OMIM
300100
Alias
ABC42,ALD,ALDP,AMN,Adrenoleukodystrophy protein
Background
Antibody anti-ABCD1
Status
RUO
Note
Concentration: 2 mg/ml - Validity: 12 months.

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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