Mitochondrial Fatty Acid β-Oxidation Disorders: From Disease to Lipidomic Studies—A Critical Review

Physiological Perspectives on the Use of Triheptanoin as Anaplerotic Therapy for Long Chain Fatty Acid Oxidation Disorders

(2021) Evgenia Sklirou et al.   Frontiers in Genetics

Different Lipid Signature in Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders

(2021) Khaled I. Alatibi et al.   Cells

Lipidomic and Proteomic Alterations Induced by Even and Odd Medium-Chain Fatty Acids on Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders

(2021) Khaled I. Alatibi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Novel LC-MS tools for diagnosing inborn errors of metabolism

(2021) Eliška Ivanovová et al.   MICROCHEMICAL JOURNAL

Biochemical Markers for the Diagnosis of Mitochondrial Fatty Acid Oxidation Diseases

(2021) Pedro Ruiz-Sala et al.   Journal of Clinical Medicine

Sex‐specific perturbation of complex lipids in response to medium‐chain fatty acids in very long‐chain acyl‐CoA dehydrogenase deficiency

(2020) Khaled I. Alatibi et al.   FEBS Journal

Lipids and phenylketonuria: Current evidences pointed the need for lipidomics studies

(2020) Inês M.S. Guerra et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Clinical manifestations and management of fatty acid oxidation disorders

(2020) J. Lawrence Merritt et al.   REVIEWS IN ENDOCRINE & METABOLIC DISORDERS

Evidence that Oxidative Disbalance and Mitochondrial Dysfunction are Involved in the Pathophysiology of Fatty Acid Oxidation Disorders

(2020) Graziela Schmitt Ribas et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Update on LIPID MAPS Classification, Nomenclature and Shorthand Notation for MS-derived Lipid Structures

(2020) Gerhard Liebisch et al.   JOURNAL OF LIPID RESEARCH

Rhomboid distorts lipids to break the viscosity-imposed speed limit of membrane diffusion

(2019) Alex J. B. Kreutzberger et al.   SCIENCE

An update on lipid oxidation and inflammation in cardiovascular diseases

(2019) Shanshan Zhong et al.   FREE RADICAL BIOLOGY AND MEDICINE

Membrane Lipid Composition: Effect on Membrane and Organelle Structure, Function and Compartmentalization and Therapeutic Avenues

(2019) Doralicia Casares et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Clinical and biochemical outcome of patients with very long-chain acyl-CoA dehydrogenase deficiency

(2019) Valentina Rovelli et al.   MOLECULAR GENETICS AND METABOLISM

Role of Cardiolipin in Mitochondrial Function and Dynamics in Health and Disease: Molecular and Pharmacological Aspects

(2019) Giuseppe Paradies et al.   Cells

Lipidomes in health and disease: Analytical strategies and considerations

(2019) Fang Wei et al.   TRAC-TRENDS IN ANALYTICAL CHEMISTRY

Inborn Errors of Metabolism in the Era of Untargeted Metabolomics and Lipidomics

(2019) Ismail et al.   Metabolites

Lipidomics from sample preparation to data analysis: a primer

(2019) Thomas Züllig et al.   ANALYTICAL AND BIOANALYTICAL CHEMISTRY

Oxidative damage in mitochondrial fatty acids oxidation disorders patients and the in vitro effect of l-carnitine on DNA damage induced by the accumulated metabolites

(2019) Maira Silmara de Moraes et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats

(2019) Cristiane Cecatto et al.   MITOCHONDRION

Impairment of mitochondrial bioenergetics and permeability transition induction caused by major long-chain fatty acids accumulating in VLCAD deficiency in skeletal muscle as potential pathomechanisms of myopathy

(2019) Cristiane Cecatto et al.   TOXICOLOGY IN VITRO

The Influence of Dietary Fatty Acids on Immune Responses

(2019) Urszula Radzikowska et al.   Nutrients

Metabolite accumulation in VLCAD deficiency markedly disrupts mitochondrial bioenergetics and Ca2+ homeostasis in the heart

(2018) Cristiane Cecatto et al.   FEBS Journal

High vulnerability of the heart and liver to 3-hydroxypalmitic acid-induced disruption of mitochondrial functions in intact cell systems

(2018) Cristiane Cecatto et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

Mitochondrial β-oxidation of saturated fatty acids in humans

(2018) María M. Adeva-Andany et al.   MITOCHONDRION

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

(2018) Suzan J. G. Knottnerus et al.   REVIEWS IN ENDOCRINE & METABOLIC DISORDERS

Very long-/ and long Chain-3-Hydroxy Acyl CoA Dehydrogenase Deficiency correlates with deregulation of the mitochondrial fusion/fission machinery

(2018) Judith Hagenbuchner et al.   Scientific Reports

Evaluation of serum sphingolipids and the influence of genetic risk factors in age-related macular degeneration

(2018) Luciana M. Pujol-Lereis et al.   PLoS One

Mitochondrial energetics is impaired in very long-chain acyl-CoA dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers

(2018) Bianca Seminotti et al.   HUMAN MOLECULAR GENETICS

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

(2018) Kenji Yamada et al.   JOURNAL OF HUMAN GENETICS

Plasma lipidomics as a diagnostic tool for peroxisomal disorders

(2017) Katharina Herzog et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Very long-chain acyl-CoA dehydrogenase (VLCAD-) deficiency–studies on treatment effects and long-term outcomes in mouse models

(2017) Sara Tucci   JOURNAL OF INHERITED METABOLIC DISEASE

The Biochemistry and Physiology of Mitochondrial Fatty Acid β-Oxidation and Its Genetic Disorders

(2016) Sander M. Houten et al.   Annual Review of Physiology

cis-4-Decenoic and decanoic acids impair mitochondrial energy, redox and Ca 2+ homeostasis and induce mitochondrial permeability transition pore opening in rat brain and liver: Possible implications for the pathogenesis of MCAD deficiency

(2016) Alexandre Umpierrez Amaral et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Carnitine transport and fatty acid oxidation

(2016) Nicola Longo et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Combined defects in oxidative phosphorylation and fatty acid  -oxidation in mitochondrial disease

(2016) A. Nsiah-Sefaa et al.   BIOSCIENCE REPORTS

The regulation of inflammation by oxidized phospholipids

(2016) Stefan Freigang   EUROPEAN JOURNAL OF IMMUNOLOGY

Unique plasma metabolomic signatures of individuals with inherited disorders of long-chain fatty acid oxidation

(2016) Colin S. McCoin et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle

(2016) Cristiane Cecatto et al.   TOXICOLOGY IN VITRO

Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency

(2015) Fernanda Hermes Hickmann et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Sexual dimorphism of lipid metabolism in very long-chain acyl-CoA dehydrogenase deficient (VLCAD−/−) mice in response to medium-chain triglycerides (MCT)

(2015) Sara Tucci et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

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

(2015) M. Wajner et al.   BIOSCIENCE REPORTS

Lipidomics: Novel insight into the biochemical mechanism of lipid metabolism and dysregulation-associated disease

(2015) Ying-Yong Zhao et al.   CHEMICO-BIOLOGICAL INTERACTIONS

De novofatty acid biosynthesis and elongation in very long-chain acyl-CoA dehydrogenase-deficient mice supplemented with odd or even medium-chain fatty acids

(2015) Sara Tucci et al.   FEBS Journal

Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart - mitochondrial permeability transition pore opening a

(2015) Cristiane Cecatto et al.   FEBS Journal

Physiological Consequences of Compartmentalized Acyl-CoA Metabolism

(2015) Daniel E. Cooper et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Acylcarnitines—old actors auditioning for new roles in metabolic physiology

(2015) Colin S. McCoin et al.   Nature Reviews Endocrinology

Oxidized phosphatidylcholines suggest oxidative stress in patients with medium-chain acyl-CoA dehydrogenase deficiency

(2015) Lukáš Najdekr et al.   TALANTA

Analytical Lipidomics in Metabolic and Clinical Research

(2015) Tuulia Hyötyläinen et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

A short introduction to acyl-CoA dehydrogenases; deficiencies and novel treatment strategies

(2015) Martin Lund et al.   Expert Opinion on Orphan Drugs

Adaptive Lipid Packing and Bioactivity in Membrane Domains

(2015) Erdinc Sezgin et al.   PLoS One

Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: A possible role of mPTP opening as a pathomechanism in these disorders?

(2014) Anelise Miotti Tonin et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Cerebrospinal fluid ceramides from patients with multiple sclerosis impair neuronal bioenergetics

(2014) Oscar G. Vidaurre et al.   BRAIN

Pathophysiology of fatty acid oxidation disorders and resultant phenotypic variability

(2013) Simon E Olpin   JOURNAL OF INHERITED METABOLIC DISEASE

Monounsaturated 14:1n-9 and 16:1n-9 Fatty Acids but not 18:1n-9 Induce Apoptosis and Necrosis in Murine HL-1 Cardiomyocytes

(2013) Lars Hoffmann et al.   LIPIDS

Functions of plasmalogen lipids in health and disease

(2012) Nancy E. Braverman et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Disruption of redox homeostasis in cerebral cortex of developing rats by acylcarnitines accumulating in medium-chain acyl-CoA dehydrogenase deficiency

(2012) Anelise M. Tonin et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria

(2012) Anelise M. Tonin et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Fatty acids and inflammation: The cutting edge between food and pharma

(2011) Philip C. Calder   EUROPEAN JOURNAL OF PHARMACOLOGY

Toxicity of octanoate and decanoate in rat peripheral tissues: evidence of bioenergetic dysfunction and oxidative damage induction in liver and skeletal muscle

(2011) Giselli Scaini et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

Aberrant lipid metabolism disrupts calcium homeostasis causing liver endoplasmic reticulum stress in obesity

(2011) Suneng Fu et al.   NATURE

A general introduction to the biochemistry of mitochondrial fatty acid β-oxidation

(2010) Sander Michel Houten et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Newborn screening for disorders of fatty-acid oxidation: experience and recommendations from an expert meeting

(2010) Martin Lindner et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse models

(2010) Ute Spiekerkoetter et al.   JOURNAL OF INHERITED METABOLIC DISEASE

The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results

(2010) Ronald J. A. Wanders et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain

(2010) Anelise M. Tonin et al.   LIFE SCIENCES

Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brain

(2010) Anelise M. Tonin et al.   NEUROCHEMISTRY INTERNATIONAL

Plasmalogens in Biological Systems: Their Role in Oxidative Processes in Biological Membranes, their Contribution to Pathological Processes and Aging and Plasmalogen Analysis

(2009) J. Leßig et al.   CURRENT MEDICINAL CHEMISTRY

Treatment recommendations in long-chain fatty acid oxidation defects: consensus from a workshop

(2009) U. Spiekerkoetter et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Medium-chain fatty acids accumulating in MCAD deficiency elicit lipid and protein oxidative damage and decrease non-enzymatic antioxidant defenses in rat brain

(2009) Patrícia F. Schuck et al.   NEUROCHEMISTRY INTERNATIONAL

Update of the LIPID MAPS comprehensive classification system for lipids

(2008) Eoin Fahy et al.   JOURNAL OF LIPID RESEARCH

Membrane lipids: where they are and how they behave

(2008) Gerrit van Meer et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mitochondrial Fatty-Acid Oxidation Disorders

(2008) Michelle Kompare et al.   Seminars in Pediatric Neurology