PMID- 8906465
OWN - NLM
STAT- MEDLINE
DCOM- 19970225
LR  - 20231213
IS  - 1572-6495 (Print)
IS  - 1572-6495 (Linking)
VI  - 684
IP  - 1-2
DP  - 1996 Sep 20
TI  - High-performance liquid chromatographic methods for the quantification of 
      tetrahydrobiopterin biosynthetic enzymes.
PG  - 51-8
AB  - Tetrahydrobiopterin is a cofactor in hydroxylation reactions, including 
      phenylalanine 4-monooxygenase, tyrosine 3-monooxygenase, tryptophan 
      5-monooxygenase, alkyl glycol ether monooxygenase and nitric oxide synthase. 
      Determination of its biosynthesis is carried out to diagnose inherited diseases 
      leading to partial defects in tetrahydrobiopterin synthesis. In addition, 
      tetrahydrobiopterin synthesis is induced by proinflammatory cytokines, and 
      intracellular levels of tetrahydro-biopterin in many cases limit the activity of 
      tetrahydrobiopterin-dependent reactions, such as nitric oxide synthase in intact 
      cells. Biosynthesis of tetrahydrobiopterin from guanosine 5'-triphosphate (GTP) 
      requires the action of three enzymes, GTP-cyclohydrolase I (E.C. 3.5.4.16), 
      6-pyruvoyl tetrahydropterin synthase (EC, 4.6.1.10) and sepiapterin reductase 
      (E.C. 1.1.1.153). Methods for quantification of biopterin and related pteridines 
      in biological matrices by HPLC and application of these for determining the 
      activity of the three tetrahydrobiopterin biosynthetic enzymes are reviewed in 
      this article.
FAU - Werner, E R
AU  - Werner ER
AD  - Institut fur Medizinische Chemie and Piechemic, Universitat Innsbuck, Austria.
FAU - Werner-Felmayer, G
AU  - Werner-Felmayer G
FAU - Wachter, H
AU  - Wachter H
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - Netherlands
TA  - J Chromatogr B Biomed Appl
JT  - Journal of chromatography. B, Biomedical applications
JID - 9421796
RN  - 0 (Antioxidants)
RN  - 0 (Biopterins)
RN  - EC 1.1.- (Alcohol Oxidoreductases)
RN  - EC 1.1.1.153 (sepiapterin reductase)
RN  - EC 3.5.4.16 (GTP Cyclohydrolase)
RN  - EC 4.6.- (Phosphorus-Oxygen Lyases)
RN  - EC 4.6.10 (6-pyruvoyltetrahydropterin synthase)
RN  - EGX657432I (sapropterin)
SB  - IM
MH  - Alcohol Oxidoreductases/*analysis
MH  - Antioxidants/*metabolism
MH  - Biopterins/*analogs & derivatives/biosynthesis
MH  - Chromatography, High Pressure Liquid/*methods
MH  - GTP Cyclohydrolase/*analysis
MH  - *Phosphorus-Oxygen Lyases
RF  - 65
EDAT- 1996/09/20 00:00
MHDA- 1996/09/20 00:01
CRDT- 1996/09/20 00:00
PHST- 1996/09/20 00:00 [pubmed]
PHST- 1996/09/20 00:01 [medline]
PHST- 1996/09/20 00:00 [entrez]
AID - 10.1016/0378-4347(95)00507-2 [doi]
PST - ppublish
SO  - J Chromatogr B Biomed Appl. 1996 Sep 20;684(1-2):51-8. doi: 
      10.1016/0378-4347(95)00507-2.