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

Name: Mouse Anti-PEX14 Recombinant Antibody (1G12)
SKU: CBMAB-P1472-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

1G12

Application

ELISA, WB

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

More Infomation

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Isotype control

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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