The biochemistry and physiology of mitochondrial fatty acid β-oxidation and its genetic disorders

SM Houten, S Violante, FV Ventura… - Annual review of …, 2016 - annualreviews.org
Mitochondrial fatty acid β-oxidation (FAO) is the major pathway for the degradation of fatty
acids and is essential for maintaining energy homeostasis in the human body. Fatty acids …

Disorders of mitochondrial long-chain fatty acid oxidation and the carnitine shuttle

SJG Knottnerus, JC Bleeker, RCI Wüst… - Reviews in Endocrine …, 2018 - Springer
Mitochondrial fatty acid oxidation is an essential pathway for energy production, especially
during prolonged fasting and sub-maximal exercise. Long-chain fatty acids are the most …

[HTML][HTML] Urban policy interventions to reduce traffic-related emissions and air pollution: A systematic evidence map

H Khreis, KA Sanchez, M Foster, J Burns… - Environment …, 2023 - Elsevier
Background Urban areas are hot spots for human exposure to air pollution, which originates
in large part from traffic. As the urban population continues to grow, a greater number of …

FGF-21 as a biomarker for muscle-manifesting mitochondrial respiratory chain deficiencies: a diagnostic study

A Suomalainen, JM Elo, KH Pietiläinen… - The Lancet …, 2011 - thelancet.com
Background Muscle biopsy is the gold standard for diagnosis of mitochondrial disorders
because of the lack of sensitive biomarkers in serum. Fibroblast growth factor 21 (FGF-21) is …

Mitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studies

M Wajner, AU Amaral - Bioscience reports, 2016 - portlandpress.com
Mitochondrial fatty acid oxidation (FAO) plays a pivotal role in maintaining body energy
homoeostasis mainly during catabolic states. Oxidation of fatty acids requires approximately …

Clinical manifestations and management of fatty acid oxidation disorders

JL Merritt, E MacLeod, A Jurecka, B Hainline - Reviews in Endocrine and …, 2020 - Springer
Fatty acid oxidation disorders (FAOD) are a group of rare, autosomal recessive, metabolic
disorders caused by variants of the genes for the enzymes and proteins involved in the …

Nutrition in chronic liver disease: consensus statement of the Indian national association for study of the liver

P Puri, RK Dhiman, S Taneja, P Tandon, M Merli… - Journal of clinical and …, 2021 - Elsevier
Malnutrition and sarcopenia are common in patients with chronic liver disease and are
associated with increased risk of decompensation, infections, wait-list mortality and poorer …

Management and diagnosis of mitochondrial fatty acid oxidation disorders: focus on very-long-chain acyl-CoA dehydrogenase deficiency

K Yamada, T Taketani - Journal of human genetics, 2019 - nature.com
Mitochondrial fatty acid oxidation disorders (FAODs) are caused by defects in β-oxidation
enzymes, including very long-chain acyl-CoA dehydrogenase (VLCAD), trifunctional protein …

Triheptanoin versus trioctanoin for long‐chain fatty acid oxidation disorders: a double blinded, randomized controlled trial

MB Gillingham, SB Heitner, J Martin… - Journal of Inherited …, 2017 - Wiley Online Library
Background Observational reports suggest that supplementation that increases citric acid
cycle intermediates via anaplerosis may have therapeutic advantages over traditional …

[HTML][HTML] Lipid replacement therapy: a natural medicine approach to replacing damaged lipids in cellular membranes and organelles and restoring function

GL Nicolson, ME Ash - Biochimica et Biophysica Acta (BBA)-Biomembranes, 2014 - Elsevier
Abstract Lipid Replacement Therapy, the use of functional oral supplements containing cell
membrane phospholipids and antioxidants, has been used to replace damaged, usually …