CFS hydroxylase cofactor levels in some neurological diseases.

Williams, Ann C.; Levine, R.; Chase, T N; Lovenberg, Walter; Calne, D. B.

doi:10.1136/jnnp.43.8.735

Cited by 75 publications

(31 citation statements)

References 17 publications

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“…Treatment with BH4 restores hepatic metabolism of phenylalanine in these infants but neurotransmitter therapy is also required and these are serious problems in clinical management. (ii) Evidence that biopterin levels are less than normal in cerebrospinal fluid (37) and in brain autopsy tissue (38) …”

mentioning

confidence: 99%

Biosynthesis of tetrahydrobiopterin by de novo and salvage pathways in adrenal medulla extracts, mammalian cell cultures, and rat brain in vivo.

Nichol¹,

Lee²,

Edelstein³

et al. 1983

Proc. Natl. Acad. Sci. U.S.A.

107

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Communicated by George H. Hitchings, December 13, 1982 ABSTRACT Mammalian cells and tissues were found to have two pathways for the biosynthesis of tetrahydrobiopterin (BH4): (i) the conversion of GTP to BH4 by a methotrexate-insensitive de novo pathway, and (ii) the conversion of sepiapterin to BH4 by a pterin salvage pathway dependent on dihydrofolate reductase (5,6,7,8-tetrahydrofolate:NADP+ oxidoreductase, EC 1.5.1.3) activity. In a Chinese hamster ovary cell mutant lacking dihydrofolate reductase (DUKX-BII), endogenous formation of BH4 proceeds normally but, unlike the parent cells, these cells or extracts ofthem do not convert sepiapterin or 7,8-dihydrobiopterin to BH4. KB cells, which do not contain detectable levels of GTP cyclohydrolase or BH4 but do contain dihydrofolate reductase, readily convert sepiapterin to BH4 and this conversion is completely prevented by methotrexate. In supernatant fractions of bovine adrenal medulla, the conversion of sepiapterin to BH4 is completely inhibited by methotrexate. Similarly, this conversion in rat brain in vivo is methotrexate-sensitive. Sepiapterin and 7,8-dihydrobiopterin apparently do not enter the de novo pathway of BH4 biosynthesis and may be derived from labile intermediates which have not yet been characterized.

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confidence: 99%

Biosynthesis of tetrahydrobiopterin by de novo and salvage pathways in adrenal medulla extracts, mammalian cell cultures, and rat brain in vivo.

Nichol¹,

Lee²,

Edelstein³

et al. 1983

Proc. Natl. Acad. Sci. U.S.A.

107

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“…These enzymes play a rate-determining role in in vivo amine (brain transmitters such as catecholamine and indole amine) biosynthetic pathways; thus, BH 4 itself is thought to exert overall control during in vivo amine synthesis activity (2). In recent years, the relationship between disorders triggered by in vivo amine concentration and BH 4 has been examined; consequently, BH 4 was determined to be a suitable indicator for several diseases. BH 4 is well known in the biochemical diagnosis of Parkinson's (3) and Alzheimer's disease (4); additionally, BH 4 is an important atypical hyperphenylalaninaemia marker in newborn mass screening (5).…”

Section: Introductionmentioning

confidence: 99%

“…In recent years, the relationship between disorders triggered by in vivo amine concentration and BH 4 has been examined; consequently, BH 4 was determined to be a suitable indicator for several diseases. BH 4 is well known in the biochemical diagnosis of Parkinson's (3) and Alzheimer's disease (4); additionally, BH 4 is an important atypical hyperphenylalaninaemia marker in newborn mass screening (5). On the other hand, BH 4 is a co-enzyme of nitric oxide synthase (NOS) (6).…”

Section: Introductionmentioning

confidence: 99%

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Chemiluminescence assay for tetrahydrobiopterin based on the generation of hydrogen peroxide using isoluminol‐microperoxidase in the presence of 1‐methoxy PMS

et al. 2007

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We developed a novel highly sensitive chemiluminescence (CL) method for BH(4). The principle of the proposed method is based on active oxygen formation induced by 1-methoxy-5-methyl phenazinium methyl sulphate (1-methoxy PMS) in the presence of dissolved oxygen. Furthermore, active oxygen is determined by a CL assay involving the luminol reaction with microperoxidase. In this report, we examined the mechanism of formation and identified the reactive oxygen species derived from BH(4) employing 1-methoxy PMS. Additionally, optimum conditions for the CL assay of BH(4) were established.

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“…Additionally, deficits of THBP metabolism have been demonstrated in various types of neurological and psychiatric diseases includ ing Parkinson's disease (4, 5), familial dys tonia (6, 7), endogenous depression (8), in fantile autism (9), etc. ; and therapeutic trials with peripheral administration of THBP have been initiated in these diseases with limited success (3)(4)(5)(6)(7)(8)(9).…”

mentioning

confidence: 99%

Enhancement of ambulation-increasing effect of methamphetamine by peripherally-administered 6R-L-erythro-5,6,7,8-tetrahydrobiopterin (R-THBP) in mice.

Asami

Kuribara

1989

Jpn.J.Pharmacol

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Abstract-Behavioral effects of 6R-L-erythro-5,6,7,8-tetra hydrobiopterin (R THBP), a co-factor for tyrosine hydroxylase and tryptophan hydroxylase, were investigated by means of ambulatory activity in mice. Single administration of R THBP (50 and 100 mg/kg, s.c.) showed no significant effect on the mouse's ambulatory activity for 5 hr. The ambulation-increasing effect of methamphetamine (2 mg/kg, s.c.) was dramatically enhanced and prolonged by the pretreatment with R-THBP (100 mg/kg, s.c.) 0, 2, 6, 12 and 24 hr before, but not 18 or 36 hr before, the methamphetamine administration. However, when combined administration of R-THBP (100 mg/kg, s.c., 2 hr before) with methamphetamine (2 mg/kg, s.c.) was repeated at intervals of 3-4 days, the enhancement by R-THBP of the methamphet amine effect was observed only in the 1st and 2nd administration, but not in the

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CFS hydroxylase cofactor levels in some neurological diseases.

Cited by 75 publications

References 17 publications

Biosynthesis of tetrahydrobiopterin by de novo and salvage pathways in adrenal medulla extracts, mammalian cell cultures, and rat brain in vivo.

Biosynthesis of tetrahydrobiopterin by de novo and salvage pathways in adrenal medulla extracts, mammalian cell cultures, and rat brain in vivo.

Chemiluminescence assay for tetrahydrobiopterin based on the generation of hydrogen peroxide using isoluminol‐microperoxidase in the presence of 1‐methoxy PMS

Enhancement of ambulation-increasing effect of methamphetamine by peripherally-administered 6R-L-erythro-5,6,7,8-tetrahydrobiopterin (R-THBP) in mice.

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