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Condition: Mitochondrial Disease

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Molecules, Vol. 26, Pages 4473: Synthesis and Biological Assessment of 4,1-Benzothiazepines with Neuroprotective Activity on the Ca2+ Overload for the Treatment of Neurodegenerative Diseases and Stroke
los Ríos In excitable cells, mitochondria play a key role in the regulation of the cytosolic Ca2+ levels. A dysregulation of the mitochondrial Ca2+ buffering machinery derives in serious pathologies, where neurodegenerative diseases highlight. Since the mitochondrial Na+/Ca2+ exchanger (NCLX) is the principal efflux pathway of Ca2+ to the cytosol, drugs capable of blocking NCLX have been proposed to act as neuroprotectants in neuronal damage scenarios exacerbated by Ca2+ overload. In our search of optimized NCLX blockers with augmented drug-likeness, we herein describe the synthesis and pharmacological characterizatio...
Source: Molecules - July 24, 2021 Category: Chemistry Authors: Luc ía Viejo Marcos Rubio-Alarc ón Raquel L. Arribas Manuel Moreno-Castro Raquel P érez-Marín Mar ía Braun-Cornejo Mart ín Estrada-Valencia Crist óbal de los Ríos Tags: Article Source Type: research

Transient Postictal Hyperglycemia as a Diagnostic Clue of Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
CONCLUSION: Seizure-induced stress hyperglycemia is uncommon in normal individuals, but such kind of energy crisis may be pronounced in patients with mitochondrial dysfunction. Early diagnosis of mitochondrial diseases-related epilepsy and hyperglycemia is crucial since certain antiepileptic drugs (ex. Valproic acid) and antihyperglycemic agents (ex. Metformin) are contraindicated in patients with mitochondrial diseases. Our findings support that transient postictal hyperglycemia may be a red flag to consider the diagnosis of MELAS.PMID:35266136
Source: Acta Neurologica Taiwanica - March 10, 2022 Category: Neurology Authors: Nai-Yi Liao Liang-Yu Lin Chien Chen Yi-Chu Liao Yi-Chung Lee Source Type: research