Chicken Peroxisomal Farnesylated Protein Polyclonal Antibody | anti-PEX19 antibody

Peroxisomal Farnesylated Protein (PXF, Peroxisomal Biogenesis Factor 19, Housekeeping Gene 33kD)

Cat. Num. AAA614245

• Gene Names: PEX19; PXF; HK33; PMP1; PMPI; PXMP1; PBD12A; D1S2223E
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by immunoaffinity chromatography.
• Synonyms: Peroxisomal Farnesylated Protein; Polyclonal Antibody; Peroxisomal Farnesylated Protein (PXF; Peroxisomal Biogenesis Factor 19; Housekeeping Gene 33kD); Anti -Peroxisomal Farnesylated Protein (PXF; anti-PEX19 antibody

• Host: Chicken
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgY
• Specificity: Recognizes human PXF. Species Crossreactivity: Mouse and rat.
• Purity/Purification: Affinity Purified; Purified by immunoaffinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Glycerol free.

• Applicable Applications for anti-PEX19 antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in ELISA and Western Blot.; Dilution: Western Blot: 1:1000-1:2000
• Immunogen: Synthetic peptide corresponding to aa1-299
• Preparation and Storage: May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Western Blot (WB)

(Western Blot Test: E coli-derived fusion protein as test antigen. Affi-anti- PXF IgY dilution: 1:2000, Goat anti-IgY-HRP dilution: 1:1000. Colorimetric method for signal development.)

Related Product Information for anti-PEX19 antibody

The covalent attachment of prenyl lipids, such as farnesyl or geranylgeranyl groups, by specific transferases is indispensable for the cellular sorting of many proteins. James et al. (1994) identified in hamster a farnesylated protein, called peroxisomal farnesylated protein or PxF, that localized to the outer surface of peroxisomes.

Product Categories/Family for anti-PEX19 antibody

Antibodies; Abs to Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 4506339
• NCBI GeneID: 5824
• NCBI Accession #: NP_002848.1
• NCBI GenBank Nucleotide #: NM_002857.3
• UniProt Accession #: P40855; Q5QNY4; Q8NI97; D3DVE7
• Molecular Weight: 32,807 Da
• NCBI Official Full Name: peroxisomal biogenesis factor 19 isoform a
• 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: Chaperone; Membrane protein, integral

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; protein targeting to peroxisome; peroxisome organization and biogenesis; peroxisome membrane biogenesis; protein import into peroxisome membrane; transmembrane transport

Disease: Peroxisome Biogenesis Disorder 12a (zellweger)