Abcam, ab211533, Anti-PMP70 antibody [CL2524]

Size: 100µL
Mouse Monoclonal PMP70 antibody. Suitable for IHC-P, ICC/IF and reacts with Human samples. Cited in 11 publications. Immunogen corresponding to Recombinant Fragment Protein within Human ABCD3 aa 400-500.
Key facts
Host species:Mouse,
Clonality:Monoclonal,
Clone number:CL2524,
Isotype:IgG1,
Carrier free:No,
Reacts with:Human,
Applications:IHC-P, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human ABCD3 aa 400-500. The exact immunogen used to generate this antibody is proprietary information.P28288,
Epitope:ab211533 binds to an epitope located within the peptide sequence GVQVIPLIPGAGEII (aa 420-434) as determined by overlapping synthetic peptides.

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
PMP70 also known as ABCD3 is a peroxisomal membrane protein with a molecular weight of approximately 70 kDa. It belongs to the ATP-binding cassette (ABC) transporter family and localizes on the peroxisomal membrane predominantly expressed in liver kidney and testes. PMP70 plays an important mechanical role in the transportation of very long-chain fatty acids (VLCFAs) across the peroxisomal membrane.
Biological function summary
This protein operates as part of a heterodimeric complex with its related transporter protein ALDP (ABCD1). PMP70 facilitates the import of VLCFAs into peroxisomes which are then subjected to β-oxidation. By doing this PMP70 contributes significantly to the metabolism of lipids maintaining internal cellular homeostasis.
Pathways
PMP70 integrates into the peroxisomal β-oxidation pathway alongside other proteins such as ALDP and ACSVL1. It ensures the proper flux of VLCFAs into peroxisomes which is essential for their breakdown and energy production. The protein also interacts with metabolic pathways linked to lipid biosynthesis and degradation illustrating its multifaceted role in cellular lipid management.
PMP70's dysfunction relates to X-linked adrenoleukodystrophy (X-ALD) a severe genetic disorder affecting the nervous system and adrenal glands. Mutations in PMP70 and related proteins particularly ALDP disrupt VLCFA degradation leading to toxic accumulation. This highlights the clinical significance of PMP70 and its potential as a therapeutic target in metabolic and neurodegenerative diseases.