Product nameAnti-PEX7 antibody [EPR7715(2)(B)]
See all PEX7 primary antibodies
DescriptionRabbit monoclonal [EPR7715(2)(B)] to PEX7
Tested applicationsSuitable for: WBmore details
Unsuitable for: Flow Cyt,ICC,IHC-P or IP
Species reactivityReacts with: Mouse, Rat, Human
Synthetic peptide within Human PEX7 aa 50-150. The exact sequence is proprietary.
- Human fetal heart lysate, Human fetal muscle lysate, MCF7 cell lysate
Storage instructionsShipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Dissociation constant (KD)KD = 1.38 x 10 -10 M Learn more about KD
Storage bufferpH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol, 0.05% BSA, 50% Tissue culture supernatant
PurityTissue culture supernatant
Our Abpromise guarantee covers the use of ab133754 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000 - 1/10000. Detects a band of approximately 36 kDa (predicted molecular weight: 36 kDa).|
FunctionBinds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.
Tissue specificityUbiquitous. Highest expression in pancreas, skeletal muscle and heart.
Involvement in diseaseDefects in PEX7 are the cause of peroxisome biogenesis disorder complementation group 11 (PBD-CG11) [MIM:614879]. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: 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. The PBD group is genetically heterogeneous with at least 13 distinct genetic groups as concluded from complementation studies.
Defects in PEX7 are the cause of rhizomelic chondrodysplasia punctata type 1 (RCDP1) [MIM:215100]. RCDP1 is characterized by rhizomelic shortening of femur and humerus, vertebral disorders, cataract, cutaneous lesions and severe mental retardation.
Defects in PEX7 are the cause of peroxisome biogenesis disorder 9B (PBD9B) [MIM:614879]. A peroxisome biogenesis disorder with unusually mild clinical and biochemical manifestations. Affected individuals manifest a variable phenotype similar to, and in some cases indistinguishable from, classic Refsum disease. Variable features include ocular abnormalities, sensorimotor neuropathy, ichthyosis, deafness, chondrodysplasia punctata without rhizomelia or growth failure.
Sequence similaritiesBelongs to the WD repeat peroxin-7 family.
Contains 6 WD repeats.
Cellular localizationPeroxisome. Cytoplasm.
- Information by UniProt
- PBD9B antibody
- PCDP1 antibody
- Peroxin 7 antibody
All lanes : Anti-PEX7 antibody [EPR7715(2)(B)] (ab133754) at 1/1000 dilution
Lane 1 : Human fetal heart lysate
Lane 2 : Human fetal muscle lysate
Lane 3 : MCF7 cell lysate
Lysates/proteins at 10 µg per lane.
All lanes : Goat anti-rabbit HRP conjugated antibody at 1/2000 dilution
Predicted band size: 36 kDa
Equilibrium disassociation constant (KD)
Learn more about KD
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ab133754 has not yet been referenced specifically in any publications.