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Tests Used for Diagnostic Purposes There are three different categories of diagnostic criteria: Genetic testing of mitochondrial DNA (mtDNA) and nuclear DNA Metabolic markers of blood, urine and cerebral spinal fluid Muscle testing assessing liver respirometry, CoQ levels, and muscle pathology Diagnosis is considered positive if two or more tests from two categories show abnormal results

Assuming that Cys desulfuration only occurs due to reactions catalyzed by CBS and CSE, kinetic simulations reported by Singh et al

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