ATP Binding Cassette Transporter D1 (ABCD1) Antibody

Este producto es parte de ABCD - ATP Binding Cassette Transporter D
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260€ (100 µl)

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935106861
info@markelab.com
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
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Product specifications

CategoryPrimary Antibodies
Immunogen TargetATP Binding Cassette Transporter D1 (ABCD1)
HostRabbit
ReactivityHuman
Recommended DilutionWB: 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.
ClonalityPolyclonal
ConjugationUnconjugated
PurificationPurified by antigen-specific affinity chromatography, followed by Protein A affinity chromatography.
Size 1100 µl
Size 2200 µl
Size 31 ml
FormLiquid
Tested ApplicationsWB, IHC, IF/ICC
Buffer0.01 M PBS, pH 7.4, containing 0.05% Proclin-300, 50% glycerol.
AvailabilityShipped within 5-7 working days.
StorageAliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
Dry IceNo
UniProt IDP33897
AliasABC42,ALD,ALDP,AMN,Adrenoleukodystrophy protein
BackgroundAntibody anti-ABCD1
StatusRUO

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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