A 10-year-old boy with dark urine and acute kidney injury: answer
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Charles Joussain & Delphine Lamireau & Caroline Espil-Taris & Valérie De Précigout & Christine Vianey-Saban & Brigitte Llanas & Jérôme Harambat
- چاپ و سال / کشور: 2011
Description
Mitochondrial fatty acid â-oxidation (FAO) is essential for energy homeostasis in skeletal muscle under conditions that require simultaneous glucose sparing and major energy supply, such as prolonged exercise or fever. The long-chain fatty acids are imported into the mitochondrial matrix by the carnitine palmitoyl transferase system. This system consists of three enzymes located in the outer and inner mitochondrial membranes. CPT I catalyzes the conversion of long-chain acylcoenzyme A derivatives (acyl-CoAs) into acylcarnitines, which pass through the inner membrane thanks to carnitine acylcarnitine translocase and are then reconverted into acyl-CoAs by CPT II (Fig. 1). The successive action of these enzymes is necessary for the entry of activated long-chain fatty acids into the mitochondria where â-oxidation of acyl-CoAs takes place. The inability of the mitochondria to use long-chain fatty acids because of CPT II deficiency diminishes energy production within the muscle and compromises sarcolemmal integrity, leading to rhabdomyolysis.
Pediatr Nephrol (2011) 26:1231–1233 DOI 10.1007/s00467-011-1767-3 Received: 20 October 2010 / Revised: 3 December 2010 / Accepted: 5 January 2011 / Published online: 29 January 2011
