Product nameAnti-PEX5/PER3 antibody
See all PEX5/PER3 primary antibodies
DescriptionRabbit polyclonal to PEX5/PER3
Tested applicationsSuitable for: IHC-P, WB, ICC/IFmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Guinea pig, Cow, Chinese hamster
- Human testis tissue; U-2 OS cells; RT-4, U-251 MG and Human liver lysates.
This product was previously labelled as PEX5
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.2
Preservative: 0.02% Sodium azide
Constituents: 59% PBS, 40% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab204444 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
|WB||1/100 - 1/250. Predicted molecular weight: 71 kDa.|
|ICC/IF||Use a concentration of 1 - 4 µg/ml.
Fixation/Permeabilization: PFA/Triton X-100.
FunctionBinds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import.
Tissue specificityDetected in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
Involvement in diseaseDefects in PEX5 are a cause of adrenoleukodystrophy neonatal (NALD) [MIM:202370]. NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long-chain fatty acids, adrenal insufficiency and mental retardation. Inheritance is autosomal recessive.
Defects in PEX5 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life.
Defects in PEX5 may be a cause of infantile Refsum disease (IRD) [MIM:266510]. IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid.
Sequence similaritiesBelongs to the peroxisomal targeting signal receptor family.
Contains 7 TPR repeats.
Cellular localizationCytoplasm. 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.
- Information by UniProt
- FLJ50634 antibody
- FLJ50721 antibody
- FLJ51948 antibody
All lanes : Anti-PEX5/PER3 antibody (ab204444) at 1/100 dilution
Lane 1 : RT-4 cell lysate
Lane 2 : U-251 MG cell lysate
Lane 3 : Human plasma lysate
Lane 4 : Human liver lysate
Predicted band size: 71 kDa
Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized U-2 OS cells labeling PEX5/PER3 with ab204444 at 4µg/ml (green).
Immunohistochemical analysis of paraffin-embedded Human testis tissue labeling PEX5/PER3 with ab204444 at 1/50 dilution.
ab204444 has not yet been referenced specifically in any publications.