Peroxisomal Biogenesis Factor 19, Recombinant Protein

PEX19 Human; Peroxisomal Biogenesis Factor 19 Human Recombinant; Peroxisomal biogenesis factor 19; Peroxisomal farnesylated protein; HK33; 33kDa housekeeping protein; PXF; PMP1; PXMP1; Peroxin-19; D1S2223E; FLJ55296

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

E Coli

Greater than 90% as determined by SDS-PAGE.

The PEX19 solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0) and 10% glycerol.
Sterile Filtered colorless solution.

Small volumes of PEX19 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.

Description: PEX19 Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 316 amino acids (1-296) and having a molecular mass of 34.6 kDa.The PEX19 is fused to a 20 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.

RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins

– Da

peroxisomal biogenesis factor 19

peroxisomal biogenesis factor 19; 33 kDa housekeeping protein; housekeeping gene, 33kD; peroxin-19; peroxisomal farnesylated protein

Peroxisomal biogenesis factor 19

HK33; PXF  [Similar Products]

PEX19_HUMAN

This gene is necessary for early peroxisomal biogenesis. It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. These disorders have at least 14 complementation groups, with more than one phenotype being observed for some complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of Zellweger syndrome (ZWS), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2010]

PEX19: Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53. Defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14); also known as PBD-CGJ. 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 PEX19 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. Belongs to the peroxin-19 family. 5 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Chaperone

Chromosomal Location of Human Ortholog: 1q23.2

Cellular Component: nucleoplasm; peroxisomal membrane; protein complex; intracellular membrane-bound organelle; brush border membrane; cytoplasm; integral to membrane; peroxisome; cytosol; nucleus

Molecular Function: protein binding; protein N-terminus binding; ATPase binding

Biological Process: peroxisome fission; protein stabilization; protein targeting to peroxisome; peroxisome organization and biogenesis; protein import into peroxisome membrane; peroxisome membrane biogenesis; transmembrane transport

Disease: Peroxisome Biogenesis Disorder 12a (zellweger)

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.