Physiological Improvement in Patients with Charcot-Marie-Tooth Disease by Pharmacological Doses of Thiamine Correlates with Increased Blood Levels of Thiamine Diphosphate DOI Open Access
Artem V. Artiukhov, Olga N. Solovjeva, Н. В. Балашова

et al.

Published: Nov. 10, 2023

Charcot-Marie-Tooth (CMT) neuropathy is a polygenic disorder of peripheral nerves with no effective cure. Thiamine (vitamin B1) neurotropic compound improving neuropathies. Our study shows therapeutic potential daily oral administration thiamine or its membrane-penetrating form sulbutiamine in CMT patients, by following their physiological performance along the blood levels coenzyme (thiamine diphosphate, ThDP), endogenous transketolase (TK) activity (holoTK), total TK (holoTK and apoTK) saturation ThDP (holoTK/(holoTK+apoTK)). The patients exhibit significant positive correlation hand grip strength. Both may decrease minor fraction apoenzyme, shifting activities to lower limit 0.03-0.04 U/mL. Although average ThDP, holoTK apoTK do not significantly differ between control groups, high interpersonal variations activation are inherent control, but CMT, subjects. difference associated statistically subjects only. Gender dependence negative correlations reversal vs. controls shown. Thus, benefits pharmacological doses linked changes thiamine-dependent metabolic network disease healthy state.

Language: Английский

Physiological Improvement in Patients with Charcot-Marie-Tooth Disease by Pharmacological Doses of Thiamine Correlates with Increased Blood Levels of Thiamine Diphosphate DOI Open Access
Artem V. Artiukhov, Olga N. Solovjeva, Н. В. Балашова

et al.

Published: Nov. 10, 2023

Charcot-Marie-Tooth (CMT) neuropathy is a polygenic disorder of peripheral nerves with no effective cure. Thiamine (vitamin B1) neurotropic compound improving neuropathies. Our study shows therapeutic potential daily oral administration thiamine or its membrane-penetrating form sulbutiamine in CMT patients, by following their physiological performance along the blood levels coenzyme (thiamine diphosphate, ThDP), endogenous transketolase (TK) activity (holoTK), total TK (holoTK and apoTK) saturation ThDP (holoTK/(holoTK+apoTK)). The patients exhibit significant positive correlation hand grip strength. Both may decrease minor fraction apoenzyme, shifting activities to lower limit 0.03-0.04 U/mL. Although average ThDP, holoTK apoTK do not significantly differ between control groups, high interpersonal variations activation are inherent control, but CMT, subjects. difference associated statistically subjects only. Gender dependence negative correlations reversal vs. controls shown. Thus, benefits pharmacological doses linked changes thiamine-dependent metabolic network disease healthy state.

Language: Английский

Citations

1