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Journal №8 for 2013 г.
Article in number:
Significance of determination of enzyme tyrosinhydroxilase in pathogenesis of dystonia responsive to dopa
Keywords: tyrosinhydroxilase GTP cyclohydrase dopamine dopa-responsive dystonia dopa-no-responsive dystonia tetrahydrobiopterin autosome-recessive and autosome-dominante inheritance
Authors:
L.R. Grinio, M.F. Mineeva, L.D. Karpova
Abstract:
Authors investigated the activity of enzyme Tyrosinhydroxilase (TH) in 15 children with clinic picture Segava disease (dystonia reacted dopha-DRD) before, during and after the treatment L-dopha (nacom). The activity of TH was determinate by the .new spectrophotometric method, which was worked by Mineeva, for the patients blood. This method permitted to reveal zero activity of TH before the treatment in the patients with DPD. The forms of TH were also were changed: TH1 and TH2 were absent, TH4 was expressed. During the treatment TH3 and TH4 were typical. Comparing these results studying of TH with the investigation in the patients with DNPD, it is possible to mark the great difference. In last group the activity of TH before the treatment was expressed and all 4 forms were presented. This fact may be useful as criterion for differentiation these two forms. Abolition of the treatment with nacom restored the clinic picture of immobility and changes of TH, this fact point out the disturbances in the dophamine metabolism in the patients with DPD as a reason of disease.
Pages: 64-68
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