Peroxisome biogenesis factor 2 (Pex2) Recombinant Protein | Pex2 recombinant protein
Recombinant Mouse Peroxisome biogenesis factor 2 (Pex2)
NCBI and Uniprot Product Information
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: Motility/polarity/chemotaxis; Membrane protein, multi-pass; Cell development/differentiation; Ubiquitin conjugating system; Membrane protein, integral
Cellular Component: peroxisomal membrane; integral to peroxisomal membrane; membrane; integral to membrane; peroxisome
Molecular Function: zinc ion binding; metal ion binding
Biological Process: nervous system development; bile acid biosynthetic process; cholesterol homeostasis; fatty acid beta-oxidation; protein destabilization; very-long-chain fatty acid metabolic process; peroxisome organization and biogenesis; negative regulation of fibroblast proliferation; 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
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Product Notes
The Pex2 pex2 (Catalog #AAA1241377) is a Recombinant Protein produced from E Coli or Yeast or Baculovirus or Mammalian Cell and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 1-305. The amino acid sequence is listed below: MAAREESTQS ANRVLRISQL DALELNKALE QLVWSQFTQC FHGFKPGLLA RFEPEVKAFL WLFLWRFTIY SKNATVGQSV LNIQHKNDSS PNPVYQPPSK NQKLLYAVCT IGGRWLEERC YDLFRNRHLA SFGKAKQCMN FVVGLLKLGE LMNFLIFLQK GKFATLTERL LGIHSVFCKP QNMREVGFEY MNRELLWHGF AEFLIFLLPL INIQKLKAKL SSWCTLCTGA AGHDSTLGSS GKECALCGEW PTMPHTIGCE HVFCYYCVKS SFLFDIYFTC PKCGTEVHSV QPLKAGIQMS EVNAL. It is sometimes possible for the material contained within the vial of "Peroxisome biogenesis factor 2 (Pex2), Recombinant Protein" 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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