Rabbit anti-Human, Mouse PEX14 Polyclonal Antibody | anti-PEX14 antibody

PEX14, ID (PEX14, Peroxisomal membrane protein PEX14, PTS1 receptor-docking protein, Peroxin-14, Peroxisomal membrane anchor protein PEX14) (FITC)

Cat. Num. AAA6332796

• Gene Names: PEX14; NAPP2; PBD13A; Pex14p; dJ734G22.2
• Reactivity: Human, Mouse
• Applications: Flow Cytometry, Functional Assay, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: PEX14; Polyclonal Antibody; ID (PEX14; Peroxisomal membrane protein PEX14; PTS1 receptor-docking protein; Peroxin-14; Peroxisomal membrane anchor protein PEX14) (FITC); Peroxisomal membrane anchor protein PEX14; anti-PEX14 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-PEX14 antibody: Flow Cytometry (FC/FACS), FLISA, Western Blot (WB)
• Application Notes: FC/FACS: 1:10-50; FLISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: PEX14 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 120-150 amino acids from the Central region of human PEX14.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(PEX14 Antibody (Center) western blot analysis in mouse Neuro-2a cell line lysates (35ug/lane).This demonstrates the PEX14 antibody detected the PEX14 protein (arrow).)

Flow Cytometry (FC/FACS)

(PEX14 Antibody (Center) flow cytometric analysis of Neuro-2a cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated donkey-anti-rabbit secondary antibodies were used for the analysis.)

Related Product Information for anti-PEX14 antibody

This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome.

Product Categories/Family for anti-PEX14 antibody

Antibodies; Receptors

References

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010). Macgregor, S., et al. Hum. Mol. Genet. 19(13):2716-2724(2010). Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010): Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009). Shiozawa, K., et al. J. Biol. Chem. 284(37):25334-25342(2009).

NCBI and Uniprot Product Information

• NCBI GI #: 4758896
• NCBI GeneID: 5195
• NCBI Accession #: NP_004556.1
• NCBI GenBank Nucleotide #: NM_004565.2
• UniProt Accession #: O75381; Q8WX51; B2R7N1; B3KML6; B7Z1N2
• Molecular Weight: 41,237 Da
• NCBI Official Full Name: peroxisomal membrane protein PEX14
• NCBI Official Synonym Full Names: peroxisomal biogenesis factor 14
• NCBI Official Symbol: PEX14
• NCBI Official Synonym Symbols: NAPP2; PBD13A; Pex14p; dJ734G22.2
• NCBI Protein Information: peroxisomal membrane protein PEX14; peroxin-14; PTS1 receptor docking protein; PTS1 receptor-docking protein; NF-E2 associated polypeptide 2; peroxisomal membrane anchor protein PEX14; peroxisomal membrane anchor protein Pex14p
• UniProt Protein Name: Peroxisomal membrane protein PEX14
• Protein Family: Peroxisomal membrane protein
• UniProt Gene Name: PEX14
• UniProt Entry Name: PEX14_HUMAN

NCBI Description

This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome. [provided by RefSeq, Jul 2008]

Uniprot Description

PEX14: Component of the peroxisomal translocation machinery with PEX13 and PEX17. Interacts with both the PTS1 and PTS2 receptors. Binds directly to PEX17. Defects in PEX14 are the cause of peroxisome biogenesis disorder complementation group K (PBD-CGK). PBD-CGK is a peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: 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 (PBD- ZSS). Defects in PEX14 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-14 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Adaptor/scaffold

Chromosomal Location of Human Ortholog: 1p36.22

Cellular Component: peroxisomal membrane; protein complex; intracellular membrane-bound organelle; membrane; nucleolus; integral to membrane; peroxisome; intracellular; nucleus

Molecular Function: protein binding; microtubule binding; beta-tubulin binding; protein N-terminus binding; transcription corepressor activity; receptor binding

Biological Process: peroxisome organization and biogenesis; negative regulation of transcription factor activity; protein import into peroxisome matrix; protein complex assembly; negative regulation of protein binding; negative regulation of transcription, DNA-dependent; protein import into peroxisome matrix, translocation; protein homooligomerization

Disease: Peroxisome Biogenesis Disorder 13a (zellweger)