ATP Synthase Deficiency due to TMEM70 Mutation Leads to Ultrastructural Mitochondrial Degeneration and Is Amenable to Treatment

Table 2

Anaplerotic therapy.

Substance	Individual dose and frequency	Application
Sodium citrate 3.13%	3 500 mg d ()	p.o. (i.v.)
Glutamine	3 500 mg d	i.v.
Sodium succinate	3 100 mg d	i.v.
Sodium aspartate	3 100 mg d	i.v.
L-Carnitine	3 100 mg/kg body weight d	p.o.
Coenzyme Q10	3 100 mg/kg body weight d	p.o.
Vitamin C	1 10 mg/kg body weight d	p.o.

(): dosage control via urine pH; target urine pH should be above pH = 7.