The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. 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 of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Oct 2008]

FLJ50634;
FLJ50721;
FLJ51948;
Peroxin 5;
Peroxin-5;
Peroxisomal biogenesis factor 5;
Peroxisomal C terminal targeting signal import receptor;
Peroxisomal C-terminal targeting signal import receptor;
Peroxisomal targeting signal 1 receptor;
Peroxisome receptor 1;
pex5;
PEX5_HUMAN;
PTS1 BP;
PTS1 receptor;
PTS1-BP;
PTS1R;
PXR1.

Mouse,Human,Rat,Dog,Pig,Cow,Horse,Sheep

IHC,IF,ICC

IHC 1:100-500
IF 1:100-500
ICC 1:100-1:400

71kD

5830

P50542

https://www.uniprot.org/uniprotkb/P50542

Cytoplasm. Peroxisome membrane. Its distribution appears to be dynamic. It is probably a cycling receptor found mainly in the cytoplasm and as well associated to the peroxisomal membrane through a docking factor.

Detected in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.

KLH conjugated synthetic peptide derived from human PEX5

Rabbit

Polyclonal

IgG

affinity purified by Protein A

Liquid

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.