Finally, protein levels of liver fatty acid binding protein (L-FABP), sterol carrier protein-2 (SCP-2), acyl CoA
binding protein (ACBP), peroxisome proliferator activated receptor (PPAR[alpha]), liver X receptor-[alpha] (LXR[alpha]), carbohydrate response element binding protein (ChREBP), and sterol response element binding protein (SREBP) were determined by western blotting as described .
Transcription regulation of peroxisomal fatty acyl CoA
oxidase and enoy-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase in rat liver by peroxisome proliferators.
Well-known mitochondrial diseases of children are Leigh's syndrome, cardiomyopathty, and medium-chain acyl CoA
dehydrogenase (MCAD) deficiency Typical mitochondrial diseases of young adults include Leber's hereditary optic neuropathy and Kearns-Sayre syndrome.
Luca's parents knew he was at high risk of developing the rare MCAD (Medium-chain acyl CoA
dehydrogenase), because another family member had died of the condition.
Whatever the precise mechanism by which PPs induce rodent liver cancer, rodent liver peroxisome proliferation, induction of the peroxisomal gene acyl CoA
oxidase (ACO) (23), hypertrophy (24), and carcinogenicity (25) are all mediated through activation of the peroxisome proliferator-activated receptor (PPAR[alpha]).
There are two isoforms of the enzyme (E.C.184.108.40.206)--the carnitine palmitoyl transferase (CPT), which is located at the inner side of the outer mitochondrial membrane, transesterifies the long-chain fatty acid and transports the acyl CoA
across the inner mitochondrial membrane, where CPT II is located, to break the acyl bond releasing the fatty acid, and recycling CoASH (2, 6-8).
Many disorders and syndromes were eliminated as the focus turned to something called short-chain acyl CoA
dehydrogenase deficiency, a fatty acid oxidation disorder within the larger category of mitochondrial disorders.
Luca Picone-Chiodo, who was at high risk of having MCAD (Medium-chain acyl CoA
dehydrogenase) a genetic condition that affects the metabolism, died at his family home in Huddersfield on October 16, 2009.