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PEX6 Rabbit pAb

PEX6 Rabbit pAb

     
  • 14 - PEX6 Rabbit pAb AP54907
    Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PEX6) Polyclonal Antibody, Unconjugated (AP54907) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P, IHC-F, IF
Primary Accession Q13608
Reactivity Mouse
Predicted Rat, Pig, Human, Rabbit, Horse
Host Rabbit
Clonality Polyclonal
Calculated MW 104061 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human PEX6
Epitope Specificity 751-850/980
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SUBCELLULAR LOCATION Cytoplasm. Peroxisome membrane. Note: Associated with peroxisomal membranes.
DISEASE The disease is caused by mutations affecting the gene represented in this entry. Disease description:A peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: 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 (PBD-ZSS).
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions This gene encodes a member of the AAA (ATPases associated with diverse cellular activities) family of ATPases. This member is a predominantly cytoplasmic protein, which plays a direct role in peroxisomal protein import and is required for PTS1 (peroxisomal targeting signal 1, a C-terminal tripeptide of the sequence ser-lys-leu) receptor activity. Mutations in this gene cause peroxisome biogenesis disorders of complementation group 4 and complementation group 6. [provided by RefSeq, Oct 2008]
Additional Information
Gene ID 5190
Other Names Peroxisomal ATPase PEX6, 3.6.4.-, Peroxin-6, Peroxisomal biogenesis factor 6, Peroxisomal-type ATPase 1, Peroxisome assembly factor 2, PAF-2, PEX6 {ECO:0000303|PubMed:10408779, ECO:0000312|HGNC:HGNC:8859}
Dilution IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name PEX6 {ECO:0000303|PubMed:10408779, ECO:0000312|HGNC:HGNC:8859}
Function Component of the PEX1-PEX6 AAA ATPase complex, a protein dislocase complex that mediates the ATP-dependent extraction of the PEX5 receptor from peroxisomal membranes, an essential step for PEX5 recycling (PubMed:16314507, PubMed:16854980, PubMed:21362118, PubMed:29884772). Specifically recognizes PEX5 monoubiquitinated at 'Cys-11', and pulls it out of the peroxisome lumen through the PEX2- PEX10-PEX12 retrotranslocation channel (PubMed:29884772). Extraction by the PEX1-PEX6 AAA ATPase complex is accompanied by unfolding of the TPR repeats and release of bound cargo from PEX5 (PubMed:29884772).
Cellular Location Cytoplasm, cytosol. Peroxisome membrane. Cell projection, cilium, photoreceptor outer segment. Note=Associated with peroxisomal membranes; anchored by PEX26 to peroxisome membranes (PubMed:12717447, PubMed:16854980). Localized at the base of the outer segment of photoreceptor cells (PubMed:26593283)
Tissue Location Expressed in the retina, at higher levels in the photoreceptor layer at the joint between the outer and inner segments
Research Areas

BACKGROUND

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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