What are the primary applications recommended for the Mouse Anti-ABCD1 Recombinant Antibody (V2-179051)?

This antibody is primarily recommended for Western Blot (WB), Immunofluorescence (IF), and Flow Cytometry (FCM). Its recombinant nature ensures high specificity and consistency across these applications.

What is the expected molecular weight of ABCD1 in Western Blot?

ABCD1 is typically detected around 75 kDa in Western blot analysis. However, its apparent molecular weight can sometimes vary slightly depending on post-translational modifications or specific sample preparation.

Mouse Anti-ABCD1 Recombinant Antibody (V2-179051) (CBMAB-A0287-YC)

See all ABCD1 antibodies

Compare Online Inquiry

Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

V2-179051

Application

IHC-P, WB

Immunogen

Human recombinant protein fragment corresponding to amino acids 508-745 of human ABCD1 (NP_000024) produced in E. coli

Specificity

Human

Antibody Isotype

IgG2a

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:2,000
IHC	1:150

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Supernatant

Buffer

PBS, pH7.3, 1% BSA, 50% Glycerol

Preservative

0.02% sodium azide

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Epitope

aa 508-745

More Infomation

Target

Full Name

ATP Binding Cassette Subfamily D Member 1

Introduction

ABCD1 is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN

Entrez Gene ID

215

UniProt ID

P33897

Alternative Names

ATP Binding Cassette Subfamily D Member 1; ATP-Binding Cassette, Sub-Family D (ALD), Member 1; Adrenoleukodystrophy Protein; ALDP; ALD; ATP-Binding Cassette Sub-Family D Member 1; ABC42; AMN;

Function

Plays a role in the transport of free very-long-chain fatty acids (VLCFAs) as well as their CoA-esters across the peroxisomal membrane by acting as an ATP-specific binding subunit releasing ADP after ATP hydrolysis. Thus, plays a role in regulation of VLCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation, mitochondrial function and microsomal fatty acid elongation. Involved in several processes; namely, controls the active myelination phase by negatively regulating the microsomal fatty acid elongation activity and may also play a role in axon and myelin maintenance. Controls also the cellular response to oxidative stress by regulating mitochondrial function like, mitochondrial oxidative phosphorylation and depolarization. And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs (By similarity).

Biological Process

Alpha-linolenic acid metabolic process
Fatty acid beta-oxidation
Fatty acid beta-oxidation using acyl-CoA oxidase
Fatty acid elongation
Fatty acid homeostasis
Linoleic acid metabolic process
Lipid transport
Long-chain fatty acid catabolic process
Long-chain fatty acid import into peroxisome
Myelin maintenance Source: UniProtKB
Negative regulation of cytokine production involved in inflammatory response
Negative regulation of reactive oxygen species biosynthetic process
Neuron projection maintenance
Peroxisomal membrane transport
Peroxisome organization
Positive regulation of fatty acid beta-oxidation
Positive regulation of unsaturated fatty acid biosynthetic process
Protein import into peroxisome membrane
Regulation of cellular response to oxidative stress
Regulation of fatty acid beta-oxidation
Regulation of mitochondrial depolarization
Regulation of oxidative phosphorylation
Sterol homeostasis
Very long-chain fatty acid catabolic process
Very long-chain fatty-acyl-CoA catabolic process

Cellular Location

Lysosome membrane; Mitochondrion membrane; Endoplasmic reticulum membrane; Peroxisome membrane

Involvement in disease

A peroxisomal metabolic disorder characterized by progressive multifocal demyelination of the central nervous system and by peripheral adrenal insufficiency (Addison disease). It results in mental deterioration, corticospinal tract dysfunction, and cortical blindness. Different clinical manifestations exist like: cerebral childhood ALD (CALD), adult cerebral ALD (ACALD), adrenomyeloneuropathy (AMN) and 'Addison disease only' (ADO) phenotype.

PTM

Tyrosine-phosphorylated.

Hong, W., Yun, W., Choi, W., Son, D., Song, G., & You, S. (2021). Generation of a WA14 hESC sub-line carrying a hemizygous ABCD1 (C. 1696_1710 del) mutation introduced by CRISPR/Cas9 technology. Stem Cell Research, 52, 102244.

Cho, Y. K., Lee, S. Y., & Kim, S. W. (2020). Novel ABCD1 Gene Mutation in a Korean Patient with X-Linked Adrenoleukodystrophy Presenting with Addison's Disease. Endocrinology and Metabolism, 35(1), 188-191.

Richter Jr, J. E., Vadlamudi, C., Macklin, S. K., Samreen, A., Helmi, H., Broderick, D., ... & Caulfield, T. R. (2020). Characterization of a Pathogenic Variant in the ABCD1 Gene Through Protein Molecular Modeling. Case reports in genetics, 2020.

Schirinzi, T., Vasco, G., Aiello, C., Rizzo, C., Sancesario, A., Romano, A., ... & Cappa, M. (2019). Natural history of a cohort of ABCD 1 variant female carriers. European journal of neurology, 26(2), 326-332.

Wang, J., Zhu, Q., & Liu, H. (2018). A novel mutation in the ABCD1 gene of a Chinese patient with X-linked adrenoleukodystrophy: Case report. Medicine, 97(21).

Ask a question We look forward to hearing from you.

0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Have you used Mouse Anti-ABCD1 Recombinant Antibody (V2-179051)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon

$30 eGift Card
Submit a review

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Related Products

Mouse Anti-ABCD1 Recombinant Antibody (V2-179057) (CAT#: CBMAB-A0293-YC)

Rabbit Anti-ABCD1 Recombinant Antibody (V2-179060) (CAT#: CBMAB-A0296-YC)

Mouse Anti-ABCD1 Recombinant Antibody (V2-179056) (CAT#: CBMAB-A0292-YC)

Mouse Anti-ABCD1 Recombinant Antibody (V2-179059) (CAT#: CBMAB-A0295-YC)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Request bulk or custom quote Second antibody and isotype control

Contact us

Tel: (USA)
(UK)
Fax:

Global Locations

Email:

Online Inquiry