Human PEX2 ELISA Kit | PEX2 elisa kit

Human PEX2 (Peroxisomal Biogenesis Factor 2) ELISA Kit

Cat. Num. AAA2507490

• Gene Names: PEX2; PAF1; PMP3; ZWS3; PBD5A; PBD5B; PMP35; PXMP3; RNF72
• Reactivity: Human
• Synonyms: PEX2; Human PEX2 (Peroxisomal Biogenesis Factor 2) ELISA Kit; PEX2 elisa kit

• Reactivity: Human
• Specificity: This kit recognizes natural and recombinant Human PEX2. No significant cross-reactivity or interference between Human PEX2 and analogues was observed.
• Sequence Length: 305

• Samples: Serum, Plasma, Biological Fluids
• Assay Type: Sandwich
• Detection Range: 0.313-20ng/mL
• Sensitivity: Min: 0.188ng/mL; Max: 20ng/mL
• Intended Uses: This ELISA kit applies to the in vitro quantitative determination of Human PEX2 concentrations in serum, plasma and other biological fluids.
• Preparation and Storage: Store at 4 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for PEX2 elisa kit

Principle of the assay: This ELISA kit uses Sandwich-ELISA as the method. The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to PEX2. Standards or samples are added to the appropriate micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibody specific for PEX2 and Avidin-Horseradish Peroxidase (HRP) conjugate is added to each micro plate well successively and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain PEX2, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color turns yellow. The optical density (OD) is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of PEX2. You can calculate the concentration of PEX2 in the samples by comparing the OD of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 121114290
• NCBI GeneID: 5828
• NCBI Accession #: NP_001073336.1
• NCBI GenBank Nucleotide #: NM_001079867.1
• UniProt Accession #: P28328; Q567S6; Q9BW41
• Molecular Weight: 34,843 Da
• NCBI Official Full Name: peroxisome biogenesis factor 2
• NCBI Official Synonym Full Names: peroxisomal biogenesis factor 2
• NCBI Official Symbol: PEX2
• NCBI Official Synonym Symbols: PAF1; PMP3; ZWS3; PBD5A; PBD5B; PMP35; PXMP3; RNF72
• NCBI Protein Information: peroxisome biogenesis factor 2; 35 kDa peroxisomal membrane protein; RING finger protein 72; peroxisomal membrane protein 3, 35kDa; peroxisome assembly factor 1; peroxisome assembly factor-1
• UniProt Protein Name: Peroxisome biogenesis factor 2
• Protein Family: Peroxisome biogenesis factor
• UniProt Gene Name: PEX2
• UniProt Synonym Gene Names: PAF1; PMP3; PMP35; PXMP3; RNF72; PAF-1
• UniProt Entry Name: PEX2_HUMAN

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: Cell development/differentiation; Motility/polarity/chemotaxis; Membrane protein, multi-pass; Ubiquitin conjugating system; Membrane protein, integral

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; 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

Disease: Peroxisome Biogenesis Disorder 5a (zellweger); Peroxisome Biogenesis Disorder 5b