Mouse Monoclonal PMP70 antibody. Suitable for IHC-P, ICC/IF and reacts with Human samples. Cited in 9 publications. Immunogen corresponding to Recombinant Fragment Protein within Human ABCD3 aa 400-500.
pH: 7.2
Preservative: 0.02% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine)
IHC-P | ICC/IF | |
---|---|---|
Human | Tested | Tested |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info 1/200.00000 - 1/500.00000 | Notes Perform heat-mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info 2.00000-10.00000 µg/mL | Notes - |
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Broad substrate specificity ATP-dependent transporter of the ATP-binding cassette (ABC) family that catalyzes the transport of long-chain fatty acids (LCFA)-CoA, dicarboxylic acids-CoA, long-branched-chain fatty acids-CoA and bile acids from the cytosol to the peroxisome lumen for beta-oxydation (PubMed:11248239, PubMed:24333844, PubMed:25168382, PubMed:29397936). Has fatty acyl-CoA thioesterase and ATPase activities (PubMed:29397936). Probably hydrolyzes fatty acyl-CoAs into free fatty acids prior to their ATP-dependent transport into peroxisomes (By similarity). Thus, play a role in regulation of LCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation (PubMed:24333844, PubMed:25944712).
PMP70, PXMP1, ABCD3, ATP-binding cassette sub-family D member 3, 70 kDa peroxisomal membrane protein
Mouse Monoclonal PMP70 antibody. Suitable for IHC-P, ICC/IF and reacts with Human samples. Cited in 9 publications. Immunogen corresponding to Recombinant Fragment Protein within Human ABCD3 aa 400-500.
pH: 7.2
Preservative: 0.02% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine)
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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.
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.
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.
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Immunofluorescent analysis of paraformalin-fixed, Triton X-100 permeabilized U251 cells labeling PMP70 with ab211533 at 4 μg/ml. Microtubule- and nuclear probes are visualized in red and blue respectively.
Immunofluorescent analysis of paraformalin-fixed, Triton X-100 permeabilized U2OS cells labeling PMP70 with ab211533 at 4 μg/ml dilution. Microtubule- and nuclear probes are visualized in red and blue respectively.
Immunofluorescent analysis of paraformalin-fixed, Triton X-100 permeabilized MCF7 cells labeling PMP70 with ab211533 at 4 μg/ml dilution. Microtubule- and nuclear probes are visualized in red and blue respectively.
Immunofluorescent analysis of paraformalin-fixed, Triton X-100 permeabilized A431 cells labeling PMP70 with ab211533 at 4 μg/ml dilution. Microtubule- and nuclear probes are visualized in red and blue respectively.
Immunofluorescent analysis of paraformalin-fixed, Triton X-100 permeabilized HeLa cells labeling PMP70 with ab211533 at 4 μg/ml dilution. Microtubule- and nuclear probes are visualized in red and blue respectively.
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human small intestine tissue labeling PMP70 with ab211533 at 1/200 dilution.
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human kidney tissue labeling PMP70 with ab211533 at 1/200 dilution.
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human fallopian tube tissue labeling PMP70 with ab211533 at 1/200 dilution.
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human liver tissue labeling PMP70 with ab211533 at 1/200 dilution.
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human prostate tissue labeling PMP70 with ab211533 at 1/200 dilution.
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human rectum tissue labeling PMP70 with ab211533 at 1/200 dilution.
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