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Recessive Epidemiology: 5 Arab Muslim patients Genetics: Glu138Lys mutation COX4I2 protein Expression: Lung COX4: COX activity regulation Complex IV May stimulate angiogenesis Oncogenic in colorectal cancer Hypoxia-associated upregulation: Hypoxia-induced release of mitochondrial reactive oxygen species Clinical GI: Exocrine pancreatic insufficiency Steatorrhea Malabsorption of lipid-soluble vitamins Skeletal Calvarial hyperostosis Bone age: delayed Osteopenia Blood: Dyserythropoietic, megaloblastic anemia MTDPS19: Epileptic encephalopathy + Anemia + Complex I deficiency Solute Carrier Family 25 (Mitochondrial carrier), Member 10 (SLC25A10)

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