Another study performed with transmitochondrial cybrids defective in mitochondrially-encoded ATP synthase membrane subunit 6 (MT-ATP6) and the Cox10−/− mouse model of mitochondrial myopathy has demonstrated that anaplerosis of the Krebs cycle with α-ketoglutarate, which can be obtained from glutamine and glutamate, mitigates the consequences of the mitochondrial defect [81]. The gene discussed is MT-ATP6; the disease is Mitochondrial myopathy.