Western Blot (WB)
(Western blot analysis of extracts from A549 cells, using PXMP3 antibody.)
Rabbit anti-Human PXMP3 Polyclonal Antibody | anti-PXMP3 antibody
PXMP3 Antibody
Immunohistochemistry: 1:50~1:100
Western Blot (WB)
(Western blot analysis of extracts from A549 cells, using PXMP3 antibody.)
Western Blot (WB)
(Western blot analysis of extracts from A549 cells, using PXMP3 antibody.)
Immunohistochemistry (IHC)
(Immunohistochemistry analysis of paraffin-embedded human breast carcinoma tissue using PXMP3 antibody.)
Immunohistochemistry (IHC)
(Immunohistochemistry analysis of paraffin-embedded human breast carcinoma tissue using PXMP3 antibody.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an integral peroxisomal membrane protein required for peroxisome biogenesis. The protein is thought to be involved in peroxisomal matrix protein import. Mutations in this gene result in one form of Zellweger syndrome and infantile Refsum disease. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008]
Uniprot Description
PXMP3: Somewhat implicated in the biogenesis of peroxisomes. Defects in PEX2 are the cause of peroxisome biogenesis disorder complementation group 5 (PBD-CG5); also known as PBD-CGF. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Defects in PEX2 are a cause of Zellweger syndrome (ZWS). ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life. Defects in PEX2 are a cause of infantile Refsum disease (IRD). IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid. Belongs to the pex2/pex10/pex12 family.
Protein type: Ubiquitin conjugating system; Membrane protein, multi-pass; Motility/polarity/chemotaxis; Membrane protein, integral; Cell development/differentiation
Chromosomal Location of Human Ortholog: 8q21.1
Cellular Component: peroxisomal membrane; integral to peroxisomal membrane; membrane
Molecular Function: protein binding; zinc ion binding
Biological Process: bile acid biosynthetic process; cholesterol homeostasis; fatty acid beta-oxidation; protein destabilization; negative regulation of fibroblast proliferation; very-long-chain fatty acid metabolic process; peroxisome organization and biogenesis; regulation of cholesterol biosynthetic process; protein import into peroxisome matrix; neuron migration; negative regulation of transcription from RNA polymerase II promoter; negative regulation of epithelial cell proliferation
Disease: Peroxisome Biogenesis Disorder 5a (zellweger); Peroxisome Biogenesis Disorder 5b
Research Articles on PXMP3
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Product Notes
The PXMP3 pex2 (Catalog #AAA9401101) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PXMP3 Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's PXMP3 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC). Western blotting: 1:500~1:3000 Immunohistochemistry: 1:50~1:100. Researchers should empirically determine the suitability of the PXMP3 pex2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PXMP3, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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