The product of this gene belongs to the acyl-CoA oxidases family. It encodes the branched-chain acyl-CoA oxidase which is involved in the degradation of long branched fatty acids and bile acid intermediates in peroxisomes. Deficiency of this enzyme results in the accumulation of branched fatty acids and bile acid intermediates, and may lead to Zellweger syndrome, severe mental retardation and death in children.
Synthetic peptide, C-QWAQKSPTNTQENP, representing the internal region (near the C Terminus) of the human sequence according to NP_003491.1
Isotype:
IgG
Epitope:
QWAQKSPTNTQENP
Species Reactivity:
Reacts with Human. Other species have not been tested.
Homology:
Human
Uses:
Peptide ELISA and Western Blot
In WB approx 75kDa band observed in Human Liver lysates (calculated MW of 76.8kDa according to NP_003491.1).
An additional band of 37kDa was consistently observed, however this band was not blocked by the immunizing peptide and it is therefore a non-specific signal. Other applications have not been tested.
Dilutions:
Suggested working dilutions * ELISA 1:4000,
Western Blot 0.5-1.5ug/ml * Investigator should determine optimal working dilutions.
Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
Limitations:
This product is for research use only and is not approved for use in humans or in clinical diagnosis. This product is guaranteed for 6 months from date of receipt.
General References:
Baumgart E, Vanhooren JC, Fransen M, Marynen P, Puype M, Vandekerckhove J, Leunissen JA, Fahimi HD, Mannaerts GP, van Veldhoven PP. Molecular characterization of the human peroxisomal branched-chain acyl-CoA oxidase: cDNA cloning, chromosomal assignment, tissue distribution, and evidence for the absence of the protein in Zellweger syndrome.Proc Natl Acad Sci U S A. 1996 Nov 26;93(24):13748-53. PMID: 8943006
Gene Id:
8309
Gene System:
ACOX2
NB100-57853 (0.5?g/ml) staining of Human Liver lysate (35?g protein in RIPA buffer) with (B) and without (A) blocking with the immunising peptide. Primary incubation was 1 hour. Detected by chemiluminescence.