Peroxisomal Biogenesis Factor 2 (PEX2), ELISA Kit

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

Chromosome: 8; NC_000008.11 (76980258..77001044, complement). Location: 8q21.1

Store all reagents at 2-8 degree C

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Small volumes of PEX2 elisa kit 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.

MBS9307331 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Peroxisomal Biogenesis Factor 2 (PEX2) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing PEX2. The ELISA analytical biochemical technique of the MBS9307331 kit is based on PEX2 antibody-PEX2 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PEX2 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PEX2. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

34,843 Da

peroxisomal biogenesis factor 2

peroxisome biogenesis factor 2; RING finger protein 72; peroxisome assembly factor 1; peroxisome assembly factor-1; 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: Cell development/differentiation; Membrane protein, multi-pass; Motility/polarity/chemotaxis; Membrane protein, integral; Ubiquitin conjugating system

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; very-long-chain fatty acid metabolic process; negative regulation of fibroblast proliferation; 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

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