Rabbit anti-Human PXMP3 Polyclonal Antibody | anti-PXMP3 antibody

PXMP3 Antibody

Cat. Num. AAA9401101

• Gene Names: PEX2; PAF1; PMP3; ZWS3; PBD5A; PBD5B; PMP35; PXMP3; RNF72
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
• Synonyms: PXMP3; Polyclonal Antibody; PXMP3 Antibody; Peroxisome assembly factor 1; PAF-1; Peroxin-2; Peroxisomal membrane protein 3; 35 kDa peroxisomal membrane protein; anti-PXMP3 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous levels of total PXMP3 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
• Form/Format: Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 305

• Applicable Applications for anti-PXMP3 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Western blotting: 1:500~1:3000; Immunohistochemistry: 1:50~1:100
• Immunogen Type: Peptide
• Immunogen Description: Synthesized peptide derived from N-terminal of human PXMP3.
• Target Name: PXMP3
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of extracts from A549 cells, using PXMP3 antibody.)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis of paraffin-embedded human breast carcinoma tissue using PXMP3 antibody.)

Related Product Information for anti-PXMP3 antibody

Somewhat implicated in the biogenesis of peroxisomes.Shimozawa N., Science 255:1132-1134(1992).Biermanns M., Biochem. Biophys. Res. Commun. 273:985-990(2000).Patarca R., FEBS Lett. 312:1-2(1992)

Product Categories/Family for anti-PXMP3 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 4506343
• NCBI GeneID: 5828
• NCBI Accession #: NP_000309.1
• NCBI GenBank Nucleotide #: NM_000318.2
• 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
• UniProt Protein Name: Peroxisome biogenesis factor 2
• 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: Ubiquitin conjugating system; Membrane protein, multi-pass; Motility/polarity/chemotaxis; Membrane protein, integral; 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; very-long-chain fatty acid metabolic process; 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