Recombinant Peroxisome biogenesis factor 2 (PEX2); Peroxisome biogenesis factor 2; 35 kDa peroxisomal membrane protein Peroxin-2 Peroxisomal membrane protein 3 Peroxisome assembly factor 1; PAF-1 RING finger protein 72

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 8; NC_000008.10 (77892494..77913280, complement). Location: 8q21.1

E Coli or Yeast or Baculovirus or Mammalian Cell

>=90% (lot specific)

Liquid containing glycerol

This protein contains an N-terminal tag and may also contain a C-terminal tag. Tag types are determined by various factors including tag-protein stability, please inquire for tag information.

Sterile filter available upon request.

Low endotoxin available upon request.

Store at -20 degree C. For extended storage, store at -20 or -80 degree C.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of PEX2 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

34,843 Da[Similar Products]

peroxisomal biogenesis factor 2

peroxisomal biogenesis factor 2; RING finger protein 72; peroxisome assembly factor 1; peroxisome assembly factor-1; peroxisome biogenesis factor 2; 35 kDa peroxisomal membrane protein; peroxisomal membrane protein 3, 35kDa

Peroxisome biogenesis factor 2

PAF1; PMP3; PMP35; PXMP3; RNF72; PAF-1??[Similar Products]

PEX2_HUMAN

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]

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: Membrane protein, integral; Motility/polarity/chemotaxis; Ubiquitin conjugating system; Membrane protein, multi-pass; 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; peroxisome organization and biogenesis; very-long-chain fatty acid metabolic process; 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

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.