What are Mitochondrial Disorders?
Mitochondria, often called the “powerhouses” of cells, are small structures within cells that provide the energy for all cellular activity. When mitochondria do not function properly they are unable to provide the necessary energy for the cell and disease can develop. Some of the most well known mitochondrial disorders are: • MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes); • MERRF (Myoclonic Epilepsy Ragged Red Fibers); • NARP (Neuropathy; Ataxia; Retinitis Pigmentosa); • MNGIE (Myoneurogenic Gastrointestinal Encephalopathy); • LHON (Leber’s Hereditary Optic Neuropathy); • KSS- Kearns Sayre Syndrome; and • Leigh’s Syndrome. The most common symptoms of mitochondrial disorders are: • muscle weakness, • exercise intolerance, • fatigue, • cognitive difficulties, and • neurological problems. The primary reason for poorly functioning mitochondria is the presence of mitochondrial DNA (mtDNA) mutations. All cells, except for egg and sperm cells, have two cop
