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Mouse Anti-PEX14 Recombinant Antibody (1G12) (CBMAB-P1472-YC)

Provided herein is a Mouse monoclonal antibody against Human Peroxisomal Biogenesis Factor 14. The antibody can be used for immunoassay techniques, such as ELISA, WB.
See all PEX14 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
1G12
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
PEX14 (NP_004556.1, 293-375 aa) partial recombinant protein with GST tag. Immunogen sequence: LGPQEEGEGV VDVKGQVRME VQGEEEKRED KEDEEDEEDD DVSHVDEEDC LGVQREDRRG GDGQINEQVE KLRRPEGASN ESE
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 293-375

Target

Full Name
peroxisomal biogenesis factor 14
Introduction
PEX14 is 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.
Entrez Gene ID
5195
UniProt ID
O75381
Alternative Names
Peroxisomal Biogenesis Factor 14; Peroxisomal Membrane Anchor Protein PEX14; Peroxin-14; Peroxisomal Membrane Anchor Protein Pex14p; Peroxisomal Membrane Protein PEX14; NF-E2 Associated Polypeptide 2; PTS1 Receptor Docking Protein;
Function
Peroxisome membrane protein that is an essential component of the peroxisomal import machinery. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PEX5). Plays a key role for peroxisome movement through a direct interaction with tubulin.
Biological Process
Microtubule anchoringManual Assertion Based On ExperimentIDA:UniProtKB
Negative regulation of DNA-binding transcription factor activityManual Assertion Based On ExperimentIDA:UniProtKB
Negative regulation of protein bindingManual Assertion Based On ExperimentIDA:UniProtKB
Negative regulation of transcription, DNA-templatedManual Assertion Based On ExperimentIDA:UniProtKB
Peroxisome organizationISS:UniProtKB
Peroxisome transport along microtubuleManual Assertion Based On ExperimentIDA:UniProtKB
Protein import into peroxisome matrixManual Assertion Based On ExperimentIMP:UniProtKB
Protein import into peroxisome matrix, dockingManual Assertion Based On ExperimentIBA:GO_Central
Protein import into peroxisome matrix, substrate releaseManual Assertion Based On ExperimentIDA:UniProtKB
Protein import into peroxisome matrix, translocationManual Assertion Based On ExperimentIDA:UniProtKB
Protein-containing complex assemblyManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Peroxisome membrane
Involvement in disease
Peroxisome biogenesis disorder complementation group K (PBD-CGK):
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).
Peroxisome biogenesis disorder 13A (PBD13A):
A fatal peroxisome biogenesis disorder belonging to the Zellweger disease spectrum and clinically characterized by severe neurologic dysfunction with profound psychomotor retardation, severe hypotonia and neonatal seizures, craniofacial abnormalities, liver dysfunction, and biochemically by the absence of peroxisomes. Additional features include cardiovascular and skeletal defects, renal cysts, ocular abnormalities, and hearing impairment. Most severely affected individuals with the classic form of the disease (classic Zellweger syndrome) die within the first year of life.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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