Rabbit PEX19 Polyclonal Antibody | anti-PEX19 antibody

Anti-PEX19 Antibody, Rabbit Polyclonal

Cat. Num. AAA8103976

• Gene Names: PEX19; PXF; HK33; PMP1; PMPI; PXMP1; PBD12A; D1S2223E
• Reactivity: Human; Predicted to React with: Pongo abelii (Species predicted to react based on 100% sequence homology)
• Applications: Western Blot
• Purity: Protein A & Antigen Affinity
• Synonyms: PEX19; Polyclonal Antibody; Anti-PEX19 Antibody; Rabbit Polyclonal; PEX19 Antibody; Rabbit PAb; Antigen Affinity Purified; peroxisomal biogenesis factor 19; Anti-D1S2223E Antibody; Anti-HK33 Antibody; Anti-PBD12A Antibody; Anti-PMP1 Antibody; Anti-PMPI Antibody; Anti-PXF Antibody; Anti-PXMP1 Antibody; anti-PEX19 antibody

• Host: Rabbit
• Reactivity: Human; Predicted to React with: Pongo abelii (Species predicted to react based on 100% sequence homology)
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human PEX19
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; PBS, pH7.0 with 0.03% Proclin300

• Applicable Applications for anti-PEX19 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:2000
• Immunogen: A synthetic peptide corresponding to the center region of the Human PEX19
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with a synthetic peptide corresponding to the center region of the Human PEX19, and purified by antigen affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

(Anti-PEX19 rabbit polyclonal antibody at 1:500 dilution Lane A: Jurkat Whole Cell Lysate Lysates/proteins at 30 ug per lane. Secondary Goat Anti-Rabbit IgG (H+L)/HRP at 1/10000 dilution. Developed using the ECL technique. Performed under reducing conditions. Predicted band size:33 kDa Observed band size:36 kDa)

Related Product Information for anti-PEX19 antibody

PEX19 is a chaperone and import receptor for newly synthesized, class I peroxisomal membrane proteins (PMPs). PEX19 binds these PMPs in the cytoplasm and delivers them to the peroxisome for subsequent insertion into the peroxisome membrane, indicating that there may be a PEX19 docking factor in the peroxisome membrane. The human peroxins PEX3 and PEX19 are essential for peroxisome biogenesis. They mediate the import of membrane proteins as well as the de novo formation of peroxisomes. The PEX3 membrane protein acts as a docking site for PEX19, a cytosolic chaperone for PMPs that delivers PMPs to the endoplasmic reticulum or peroxisomal membrane. PEX19 binds newly synthesized peroxisomal membrane proteins post-translationally and directs them to peroxisomes by engaging PEX3, a protein anchored in the peroxisomal membrane. After protein insertion into the lipid bilayer, PEX19 is released back to the cytosol.

References

Schmidt F, et al. (2012) The role of conserved pex3 regions in pex19-binding and peroxisome biogenesis. Traffic 13 (9): 1244-1260.McDonnell MM, et al. (2016) The early-acting peroxin pex19 is redundantly encoded, farnesylated, and essential for viability in arabidopsis thaliana. PLoS One 11 (1): e0148335.Fang Y, et al. (2004) Pex3 functions as a pex19 docking factor in the import of class i peroxisomal membrane proteins. J Cell Biol 164 (6): 863-875.Agrawal G, et al. (2017) Functional regions of the peroxin pex19 necessary for peroxisome biogenesis. J Biol Chem 292 (27): 11547-11560.

NCBI and Uniprot Product Information

• NCBI GI #: 302370946
• NCBI GeneID: 5824
• NCBI Accession #: NP_001180573.1
• NCBI GenBank Nucleotide #: NM_001193644.1
• UniProt Accession #: P40855; Q5QNY4; Q8NI97; D3DVE7
• Molecular Weight: 32,807 Da
• NCBI Official Full Name: peroxisomal biogenesis factor 19 isoform c
• NCBI Official Synonym Full Names: peroxisomal biogenesis factor 19
• NCBI Official Symbol: PEX19
• NCBI Official Synonym Symbols: PXF; HK33; PMP1; PMPI; PXMP1; PBD12A; D1S2223E
• NCBI Protein Information: peroxisomal biogenesis factor 19; peroxin-19; housekeeping gene, 33kD; 33 kDa housekeeping protein; peroxisomal farnesylated protein
• UniProt Protein Name: Peroxisomal biogenesis factor 19
• Protein Family: Peroxisomal biogenesis factor
• UniProt Gene Name: PEX19
• UniProt Synonym Gene Names: HK33; PXF
• UniProt Entry Name: PEX19_HUMAN

NCBI Description

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]

Uniprot Description

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; nucleus; cytosol

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

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

Disease: Peroxisome Biogenesis Disorder 12a (zellweger)