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In case of early onset phenotype, which is estimated to occur in approximately 10% of disease carriers [22], patients usually show symptoms before the age of 5 [23] and are severely affected with rapid disease progression, marked muscular wasting, and weakness [24]

Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

49 , W293-w296

Within the plant, H 2 S is biologically reactive, directly incorporated into Cys