Electron transfer flavoprotein and its role in mitochondrial energy metabolism in health and disease

BJ Henriques, RKJ Olsen, CM Gomes, P Bross - Gene, 2021 - Elsevier
Electron transfer flavoprotein (ETF) is an enzyme with orthologs from bacteria to humans.
Human ETF is nuclear encoded by two separate genes, ETFA and ETFB, respectively. After …

[HTML][HTML] Redox signalling and mitochondrial stress responses; lessons from inborn errors of metabolism

RKJ Olsen, N Cornelius, N Gregersen - Journal of inherited metabolic …, 2015 - Springer
Mitochondria play a key role in overall cell physiology and health by integrating cellular
metabolism with cellular defense and repair mechanisms in response to physiological or …

[PDF][PDF] Zebrafish embryonic lipidomic analysis reveals that the yolk cell is metabolically active in processing lipid

D Fraher, A Sanigorski, NA Mellett, PJ Meikle… - Cell reports, 2016 - cell.com
The role of lipids in providing energy and structural cellular components during vertebrate
development is poorly understood. To elucidate these roles further, we visualized lipid …

Emerging roles for riboflavin in functional rescue of mitochondrial β-oxidation flavoenzymes

BJ Henriques, RK Olsen, P Bross… - Current medicinal …, 2010 - ingentaconnect.com
Riboflavin, commonly known as vitamin B2, is the precursor of flavin cofactors. It is present in
our typical diet, and inside the cells it is metabolized to FMN and FAD. As a result of their …

Evidence that oxidative disbalance and mitochondrial dysfunction are involved in the pathophysiology of fatty acid oxidation disorders

GS Ribas, CR Vargas - Cellular and Molecular Neurobiology, 2022 - Springer
Mitochondrial fatty acid β-oxidation disorders (FAODs) are a group of about 20 diseases
which are caused by specific mutations in genes that codify proteins or enzymes involved in …

Expanding role of vitamin E in protection against metabolic dysregulation: Insights gained from model systems, especially the developing nervous system of zebrafish …

B Head, MG Traber - Free Radical Biology and Medicine, 2021 - Elsevier
This review discusses why the embryo requires vitamin E (VitE) and shows that its lack
causes metabolic dysregulation and impacts morphological changes at very early stages in …

Lipid storage myopathies: current treatments and future directions

ER Vasiljevski, MA Summers, DG Little… - Progress in lipid …, 2018 - Elsevier
Lipid storage myopathies (LSMs) are a heterogeneous group of genetic disorders that
present with abnormal lipid storage in multiple body organs, typically muscle. Patients can …

How mitochondrial dysfunction affects zebrafish development and cardiovascular function: an in vivo model for testing mitochondria‐targeted drugs

BR Pinho, MM Santos, A Fonseca‐Silva… - British journal of …, 2013 - Wiley Online Library
Background and Purpose Mitochondria are a drug target in mitochondrial dysfunction
diseases and in antiparasitic chemotherapy. While zebrafish is increasingly used as a …

Disorders of flavin adenine dinucleotide metabolism: MADD and related deficiencies

M Mereis, RJA Wanders, M Schoonen… - The international journal …, 2021 - Elsevier
Multiple acyl-coenzyme A dehydrogenase deficiency (MADD), or glutaric aciduria type II
(GAII), is a group of clinically heterogeneous disorders caused by mutations in electron …

High-resolution analysis of metabolic cycles in the intertidal mussel Mytilus californianus

KM Connor, AY Gracey - American Journal of Physiology …, 2012 - journals.physiology.org
Inhabitants of the marine rocky intertidal live in an environment that alternates between
aquatic and terrestrial due to the rise and fall of the tide. The tide creates a cyclical …