Anti-PEX5 monoclonal Antibody | MB62351

Anti-PEX5 monoclonal Antibody | MB62351 | Gentaur UK, US & Europe Distribution

Host: Mouse

Reactivity: Human, Monkey, Mouse, Rat, Dog

Application: FC, IF, WB

Application Range: WB 1:500~2000, IF 1:100, FLOW 1:100

Background: 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]

Storage & Stability: PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.

Specificity: peroxisomal biogenesis factor 5

Molecular Weight: 69.7 kDa (Predicted)

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: PBD2A; PBD2B; PTS1-BP; PTS1R; PXR1; RCDP5

Immunogen: Full length human recombinant protein of human PEX5 (NP_000310) produced in HEK293T cell.

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)

Pathway: N/A