ABCD1 Antibody (1AL-2B4)

• Reactivity: Hu
• Applications: WB, ELISA, ICC/IF, IHC
• Clone: 1AL-2B4
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Unconjugated

ABCD1 Antibody (1AL-2B4) Summary

• Description: Relevant Entrez Gene Numbers: NM_000033.2
• 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.
• Isotype: IgG1
• Clonality: Monoclonal
• Host: Mouse
• Gene: ABCD1
• Purity: Unpurified

Applications/Dilutions

• Dilutions:
  • ELISA 1:500-1:5000
  • Immunocytochemistry/ Immunofluorescence 1:500-1:5000
  • Immunohistochemistry 1:500-1:5000
  • Western Blot 1:500-1:5000

Packaging, Storage & Formulations

• Storage: Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
• Buffer: Ascites
• Preservative: No Preservative
• Purity: Unpurified

Notes

Relevant Entrez Gene Numbers: NM_000033.2

Alternate Names for ABCD1 Antibody (1AL-2B4)

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

Background

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.

Limitations

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.

Bioinformatics

• Gene Symbol: ABCD1
• Uniprot:
  • P33897(Human)