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

PXMP3, ID (PEX2, PAF1, PMP3, PMP35, PXMP3, RNF72, Peroxisome biogenesis factor 2, 35kD peroxisomal membrane protein, Peroxin-2, Peroxisomal membrane protein 3, Peroxisome assembly factor 1, RING finger protein 72) (APC)

Cat. Num. AAA6339295

• Gene Names: PEX2; PAF1; PMP3; ZWS3; PBD5A; PBD5B; PMP35; PXMP3; RNF72
• Reactivity: Human, Mouse
• Applications: Immunohistochemistry, Western Blot, Flow Cytometry, Functional Assay
• Purity: Purified by Protein A and Peptide Affinity Chromatography.
• Synonyms: PXMP3; Polyclonal Antibody; ID (PEX2; PAF1; PMP3; PMP35; RNF72; Peroxisome biogenesis factor 2; 35kD peroxisomal membrane protein; Peroxin-2; Peroxisomal membrane protein 3; Peroxisome assembly factor 1; RING finger protein 72) (APC); PEX2; RING finger protein 72; anti-PXMP3 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human PXMP3. Species Crossreactivity: mouse
• Purity/Purification: Purified by Protein A and Peptide Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

• Applicable Applications for anti-PXMP3 antibody: Immunohistochemistry (IHC) Paraffin, Western Blot (WB), Flow Cytometry (FC/FACS), FLISA
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 165-194 in the central region of human PXMP3.
• Conjugate: APC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of mouse cerebellum tissue lysates (35ug/lane) usingMBS649562. PXMP3 (arrow) was detected usingMBS649562.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human hepatocarcinoma reacted withMBS649562, which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of MBS649562 for immunohistochemistry.)

Flow Cytometry (FC/FACS)

(Flow cytometric analysis of WiDr cells (right histogram) using MBS649562 compared to a negative control cell (left histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-PXMP3 antibody

Antibodies; Obesity Proteins

References

1. Steinberg, S., et.al., Mol. Genet. Metab. 83 (3), 252-263 (2004). 2. Biermanns, M., et.al., Eur. J. Cell Biol. 82 (4), 155-162 (2003).

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

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; neuron migration; protein import into peroxisome matrix; 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