ABCD1 Antibody (1AL-2B4)

Novus Biologicals | Catalog # NBP2-29709

Novus Biologicals
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Key Product Details

Species Reactivity

Human

Applications

Immunohistochemistry, Western Blot, ELISA, Immunocytochemistry/ Immunofluorescence

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG1 Clone # 1AL-2B4
View all ABCD1 Primary Antibodies »

Product Specifications

Immunogen

ALDP fragment from aa 495-648 as a fusion protein.

Specificity

Human adrenoleukodystrophy protein (ALDP). No cross-reactivity on mouse ALDP, mouse ALDRP and PMP 70. MAB2162 can be used in Western Blot, immunofluorescence or immunohistochemical studies to analyze the ALD protein in cells and tissues from normal individuals or from patients with adrenoleukodystrophy (ALD) or Zellweger syndrome and related peroxisomal diseases, and provides an excellent marker for peroxisomes(Mosser et al., 1994). About 70% of ALD male patients have no detectable ALDP in fibroblasts or leukocytes. A similar proportion of carrier females may show mosaicism for the presence or absence of ALDP.

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1

Description

Relevant Entrez Gene Numbers: NM_000033.2

Applications for ABCD1 Antibody (1AL-2B4)

Application
Recommended Usage

ELISA

1:500-1:5000

Immunocytochemistry/ Immunofluorescence

1:500-1:5000

Immunohistochemistry

1:500-1:5000

Western Blot

1:500-1:5000

Formulation, Preparation, and Storage

Purification

Unpurified

Formulation

Ascites

Preservative

No Preservative

Concentration

This product is unpurified. The exact concentration of antibody is not quantifiable.

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.

Background: ABCD1

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, GCN20, White). ABCD1 is a member of the ALD subfamily, which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. ABCD1 peroxisomal membrane protein is likely involved in the peroxisomal transport or catabolism of very long chain fatty acids. Defects in ABCD1 have been identified as the underlying cause of adrenoleukodystrophy, an X-chromosome recessively inherited demyelinating disorder of the nervous system.

Alternate Names

Adrenoleukodystrophy protein, ALDATP-binding cassette sub-family D member 1, ALDPadrenoleukodystrophy, AMNABC42, ATP-binding cassette, sub-family D (ALD), member 1

Gene Symbol

ABCD1

UniProt

P33897 (Human)

Additional ABCD1 Products

Product Documents for ABCD1 Antibody (1AL-2B4)

Certificate of Analysis

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Product Specific Notices for ABCD1 Antibody (1AL-2B4)

Relevant Entrez Gene Numbers: NM_000033.2

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Protocols

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FAQs

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