5C53.22
Coenzyme Q10 deficiency
Deficiência de coenzima Q10
CategoryDefinition
This is a deficiency in a 1,4-benzoquinone, where Q refers to the quinone chemical group, and 10 refers to the number of isoprenyl chemical subunits in its tail. This oil-soluble, vitamin-like substance is present in most eukaryotic cells, primarily in the mitochondria. It is a component of the electron transport chain and participates in aerobic cellular respiration, generating energy in the form of ATP.
Index Terms
Coenzyme Q10 deficiencyCoQ10 deficiencyEarly-onset ataxia with oculomotor apraxia and hypoalbuminaemiaAtaxia - oculomotor apraxia type 1Deafness - encephaloneuropathy - obesity - valvulopathy