[Management of the mitochondrial encephalopathies]
Related Experiment Video
Updated: Oct 12, 2026

Treating SCA1 Mice with Water-Soluble Compounds to Non-Specifically Boost Mitochondrial Function
Published on: January 22, 2017
1Servicio de Neurologia Pediátrica, Columbia Rio Grande Regional Hospital, McAllen, Texas, USA. leogarcia@pol.net
Introduction:
In the management of mitochondrial diseases, it is most important to know what mitochondrias are, how they function and the pattern of inheritance present on the different conditions affecting them.
Development:
An overview of mitochondrial function, inheritance, clinical and biochemical classification is done, reviewing the different fatty acid oxidation, pyruvic metabolism and respiratory chain defects. Classification by age of onset is also done. Approach to diagnosis, clinical aspects, laboratory findings and available treatment is discussed. Some comments about the use of a neurometabolic database are discussed, where more than 150 conditions are described in detail.
Conclusions:
By using the search capabilities of the software, a search by age of onset, symptoms, signs and laboratory findings help in reaching the most probable diagnosis in a given child, with its recommended current management.
More Related Videos
08:48Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models
Published on: June 30, 2023
08:56Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
Published on: October 10, 2025
Related Concept Videos
Mitochondrial Membranes
Electron Transport Chain: Complex I and II
ROS generation is regulated and maintained at moderate levels necessary...
ATP Synthase: Mechanism
Mitochondrial Protein Sorting
Most of these mitochondrial proteins are encoded by the nucleus and imported to the mitochondria as unfolded or loosely folded precursors. Mitochondrial precursors...
Mitochondrial Precursor Proteins
Most of the mitochondrial precursors...
Hepatic Encephalopathy