title: 6-pyruvoyl tetrahydropterin synthase from salmon liver: amino acid sequence analysis by tandem mass spectrometry.
abstract: The most frequent variant of atypical phenylketonuria, an inborn error of metabolism, is characterized by a low activity of the 6-pyruvoyl tetrahydropterin synthase. We purified and characterized this enzyme from salmon liver known to contain high levels. After digestion, peptides were sequenced by tandem mass spectrometry and/or automated Edman microsequence analysis. Both a free amine terminus and an N-acetylated amine terminus were found, indicating the presence of two isoforms. The peptide sequences determined here have a high degree of homology with the protein sequence deduced from cDNA for rat 6-pyruvoyl tetrahydropterin synthase (1), however, the amine termini of these proteins differ significantly.
authors.affiliation: Department of Pediatrics, University of Zurich, Switzerland.
authors.forename: C R
authors.initials: CR
authors.lastname: Hauer
authors.forename: W
authors.initials: W
authors.lastname: Leimbacher
authors.forename: P
authors.initials: P
authors.lastname: Hunziker
authors.forename: F
authors.initials: F
authors.lastname: Neuheiser
authors.forename: N
authors.initials: N
authors.lastname: Blau
authors.forename: C W
authors.initials: CW
authors.lastname: Heizmann
mesh_terms: D000429:Alcohol Oxidoreductases / Q000737:chemistry* / Q000235:genetics / Q000302:isolation & purification; D000595:Amino Acid Sequence; D000818:Animals; D002851:Chromatography, High Pressure Liquid; D015180:Electrophoresis, Gel, Two-Dimensional; D004591:Electrophoresis, Polyacrylamide Gel; D007202:Indicators and Reagents; D008099:Liver / Q000201:enzymology*; D013058:Mass Spectrometry; D008969:Molecular Sequence Data; D008970:Molecular Weight; D010446:Peptide Fragments / Q000302:isolation & purification; D019761:Phosphorus-Oxygen Lyases; D051381:Rats; D012474:Salmon; D012689:Sequence Homology, Nucleic Acid
chemical_list: D007202:Indicators and Reagents; D010446:Peptide Fragments; D000429:Alcohol Oxidoreductases; D019761:Phosphorus-Oxygen Lyases; C043497:6-pyruvoyltetrahydropterin synthase