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These compounds enhance the liver and gallbladder's role by decreasing fat deposits and speeding up metabolism of fat and its removal

Recessive Epidemiology: 7 families Genetics Mutations: Missense, Nonsense or Deletion Allelic disorder: Infant onset multisystem disease TMEM126B protein Location: Inner mitochondrial membrane Complex I assembly factor Forms complex with other assembly factors: NDUFAF1, ACAD9, ECSIT Clinical Onset age: 3 to 38 years Fatigue Exercise intolerance Myalgia Vomiting Weakness: Proximal Laboratory Lactate & Alanine: High in cerum Serum CK: Normal Muscle Ultrastructure: Mitochondria large & Inclusions Complex I deficiency TMEM126B variant syndrome: Infant onset multisystem syndrome Epidemiology: 1 patient Genetics Inheritance: Recessive Mutations: Homozygous Gly212Val Clinical Failure to thrive Renal failure Cardiomyopathy Respiratory failure COXPD26: CNS , Demyelinating Neuropathy + Myopathy with Exercise Intolerance (PNSED) 198 tRNA Methyltransferase 5, S