Mouse Anti-PEX3 Recombinant Antibody (3C2) (CBMAB-P1479-YC)

Name: Mouse Anti-PEX3 Recombinant Antibody (3C2)
SKU: CBMAB-P1479-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

3C2

Application

ELISA, WB

Immunogen

PEX3 (NP_003621, 271-373 aa) partial recombinant protein with GST tag. Immunogen sequence: LESPDFSTVL NTCLNRGFSR LLDNMAEFFR PTEQDLQHGN SMNSLSSVSL PLAKIIPIVN GQIHSVCSET PSHFVQDLLT MEQVKDFAAN VYEAFSTPQQ LEK

Specificity

Human

Antibody Isotype

IgG2b, 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 271-373

More Infomation

Target

Full Name

peroxisomal biogenesis factor 3

Introduction

PEX3 is involved in peroxisome biosynthesis and integrity. It assembles membrane vesicles before the matrix proteins are translocated. 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. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular 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 Zellweger syndrome (ZWS).

Entrez Gene ID

8504

UniProt ID

P56589

Alternative Names

Peroxisomal Biogenesis Factor 3; Peroxisomal Assembly Protein PEX3; Peroxin-3; Transformation-Related Protein 18; PBD10A; PBD10B; TRG18;

Function

Involved in peroxisome biosynthesis and integrity. Assembles membrane vesicles before the matrix proteins are translocated. As a docking factor for PEX19, is necessary for the import of peroxisomal membrane proteins in the peroxisomes.

Biological Process

Peroxisome organizationManual Assertion Based On ExperimentIMP:UniProtKB
Protein import into peroxisome membraneManual Assertion Based On ExperimentIMP:UniProtKB

Cellular Location

Peroxisome membrane

Involvement in disease

Peroxisome biogenesis disorder complementation group 12 (PBD-CG12):
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 10A (PBD10A):
Peroxisome biogenesis disorder 10A (PBD10A)

Topology

Cytoplasmic: 1-15
Helical: 16-36
Peroxisomal: 37-116
Helical: 117-140
Cytoplasmic: 117-140

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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)
Proteogenomic
Other Protocols

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Mouse Anti-PEX3 Recombinant Antibody (B-6) (CAT#: CBMAB-P1480-YC)

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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