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Products:Signal Transduction >> Metabolism >> Mitochondrial
Overview
Product name
HADHB peptide (2-13)
Protein description
Synthetic peptide: TILTYPFKNLPT-C, corresponding to amino acids 2-13 of Human HADHB.
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Concentration
Concentration information loading...
Sequence
TILTYPFKNL PT-C
Applications
Application notes
This peptide can be used with studies using ab10093.
Protein info
Pathway
Lipid metabolism; fatty acid beta-oxidation.
Involvement in disease
Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex.
Sequence similarities
Belongs to the thiolase family.
Cellular localization
Mitochondrion.
Target information above from: UniProt accessionP55084
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
References for HADHB peptide (2-13) (ab23180)
ab23180 has not yet been referenced specifically in any publications.
Publishing research using ab23180? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"