Background:

The product of 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 PEX3 are a cause Zellweger syndrome (ZWS).

Classification:

Primary antibody

Alternative names:

PEX3; Peroxisomal biogenesis factor 3; Peroxin-3; Peroxisomal assembly protein PEX3

Species reactivity:

Human;Mouse;Rat

Host:

Rabbit

Isotype:

IgG

Immunogen:

The antiserum was produced against synthesized peptide derived from human PEX3. AA range:12-61

Concentration:

1 mg/ml

Molecular weight:

42140

Applications:

WB, ELISA

Storage:

-20°C for one year

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