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doi: 10.1007/s10545-013-9640-0

These mitochondrial impairments not only compromise energy production but also act as pro-inflammatory stimuli, promoting chronic neuroinflammation through dysregulated mitochondrial signaling and innate immune response

OXPHOS is the biochemical process by which mitochondria produce energy in mammalian cells in the form of adenosine triphosphate (ATP)

The fishy body odor results from excessive production and excretion of trimethylamine, a metabolite of choline

However, he presented with severe neonatal onset cardiomyopathy, which seems to be part of a syndromic condition and unrelated to MCAD deficiency