Literature DB >> 2477772

Tetrahydrobiopterin administration in biopterin-deficient progressive dystonia with diurnal variation.

J K Fink1, P Ravin, C E Argoff, R A Levine, R O Brady, M Hallett, N W Barton.   

Abstract

We administered tetrahydrobiopterin (BH4) to 4 patients with progressive dystonia with diurnal variation (PDDV). One patient improved clinically. Deficient CSF concentrations of HVA and 5-HIAA were unchanged despite marked elevation of CSF biopterin concentration. Variable effectiveness of BH4 in PDDV may reflect reduced number or function of biopterin-metabolizing neurons or variable entry of BH4 into these neurons.

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Year:  1989        PMID： 2477772     DOI： 10.1212/wnl.39.10.1393

Source DB:  PubMed          Journal:  Neurology        ISSN： 0028-3878            Impact factor:   9.910


  6 in total

1.  Whole-genome sequencing for optimized patient management.

Authors:  Matthew N Bainbridge; Wojciech Wiszniewski; David R Murdock; Jennifer Friedman; Claudia Gonzaga-Jauregui; Irene Newsham; Jeffrey G Reid; John K Fink; Margaret B Morgan; Marie-Claude Gingras; Donna M Muzny; Linh D Hoang; Shahed Yousaf; James R Lupski; Richard A Gibbs
Journal:  Sci Transl Med       Date:  2011-06-15       Impact factor: 17.956

2.  Developmental susceptibility of neurons to transient tetrahydrobiopterin insufficiency and antenatal hypoxia-ischemia in fetal rabbits.

Authors:  Lei Yu; Jeannette Vásquez-Vivar; Rugang Jiang; Kehuan Luo; Matthew Derrick; Sidhartha Tan
Journal:  Free Radic Biol Med       Date:  2013-12-04       Impact factor: 7.376

Review 3.  Which neuroprotective agents are ready for bench to bedside translation in the newborn infant?

Authors:  Nicola J Robertson; Sidhartha Tan; Floris Groenendaal; Frank van Bel; Sandra E Juul; Laura Bennet; Matthew Derrick; Stephen A Back; Raul Chavez Valdez; Frances Northington; Alistair Jan Gunn; Carina Mallard
Journal:  J Pediatr       Date:  2012-02-09       Impact factor: 4.406

Review 4.  Tyrosine hydroxylase and Parkinson's disease.

Authors:  J Haavik; K Toska
Journal:  Mol Neurobiol       Date:  1998-06       Impact factor: 5.590

5.  Cerebrospinal fluid biopterin and biogenic amine metabolites during oral R-THBP therapy for infantile autism.

Authors:  H Komori; T Matsuishi; S Yamada; Y Yamashita; E Ohtaki; H Kato
Journal:  J Autism Dev Disord       Date:  1995-04

6.  Tetrahydrobiopterin in the prevention of hypertonia in hypoxic fetal brain.

Authors:  Jeannette Vásquez-Vivar; Jennifer Whitsett; Matthew Derrick; Xinhai Ji; Lei Yu; Sidhartha Tan
Journal:  Ann Neurol       Date:  2009-09       Impact factor: 10.422
  6 in total

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