Mitochondrial damage: an important feature in a number of inborn errors of metabolism?

Quantum Nondemolition Measurement of a Nonclassical State of a Massive Object.

Physical review. X·2016

Mechanically Mediated Microwave Frequency Conversion in the Quantum Regime.

Physical review letters·2016

Astrocyte NMDA receptors' activity sustains neuronal survival through a Cdk5-Nrf2 pathway.

Cell death and differentiation·2015

Successful reversal of propionic acidaemia associated cardiomyopathy: evidence for low myocardial coenzyme Q10 status and secondary mitochondrial dysfunction as an underlying pathophysiological mechanism.

Mitochondrion·2014

Mitochondrial dysfunction in the skeletal muscle of a mouse model of Rett syndrome (RTT): implications for the disease phenotype.

Mitochondrion·2014

Effect of Coenzyme Q10 supplementation on mitochondrial electron transport chain activity and mitochondrial oxidative stress in Coenzyme Q10 deficient human neuronal cells.

The international journal of biochemistry & cell biology·2014

Treatment of Fatty Acid Oxidation Disorders Today: Emerging Personalized Treatment Strategies.

Journal of inherited metabolic disease·2026

International Guideline on the Diagnosis, Treatment, and Monitoring of Long-Chain Fatty Acid Oxidation Disorders (LC-FAOD).

Journal of inherited metabolic disease·2026

L-Carnitine Prevents Lipopolysaccharide-Induced Inflammatory Response, Microglia Activation and Neuronal Damage in Cerebral Cortex, as Well as Neuromotor Development Delay in Neonatal Glutaryl-CoA Dehydrogenase Deficient (Gcdh-/-) Mice.

Journal of inherited metabolic disease·2026

Longitudinal Assessment of Cognitive Development in 23 Patients With Mucopolysaccharidosis (MPS) Type II: Results of up to 14 Years of Follow-Up.

Journal of inherited metabolic disease·2026

Distinct Urea Cycle Dysfunction Profiles Differentiate Acute Metabolic Decompensation in TMEM70 and MT-ATP6-Related Mitochondrial ATP Synthase Defects.

Journal of inherited metabolic disease·2026

Sarcopenia in Pediatric Intoxication Type Inborn Errors of Metabolism: A Frequent and Underrecognized Condition.

Journal of inherited metabolic disease·2026

Limbic-Predominant Cortical Magnetic Susceptibility Alterations Are Associated With Glycemic Dysregulation and Cognitive Performance in Type 2 Diabetes.

CNS neuroscience & therapeutics·2026

Efficacy of Dapagliflozin vs. Saroglitazar on Hepatic Steatosis and Fibrosis in Patients of Type 2 Diabetes Mellitus and Metabolic-Associated Steatotic Liver Disease (MASLD).

Indian journal of endocrinology and metabolism·2026

A 48-Month, Prospective, Multicenter, Natural History Study of Choroideremia.

Ophthalmology science·2026

Therapeutic and chemopreventive potential of melatonin in liver diseases: targeting oxidative stress and inflammatory signaling.

Frontiers in pharmacology·2026

Mitochondrial permeability transition in skeletal muscle phenocopies muscle alterations seen in cancer cachexia and other wasting conditions.

The Journal of physiology·2026

A cardiometabolic risk phenotype index integrating insulin resistance, systemic inflammation, and psychological burden for mortality risk stratification in metabolic dysfunction-associated steatotic liver disease: an exploratory longitudinal analysis of a nationally representative cohort.

Lipids in health and disease·2026

Related Experiment Videos

Mitochondrial damage: an important feature in a number of inborn errors of metabolism?

Related Concept Videos

Quantum Nondemolition Measurement of a Nonclassical State of a Massive Object.

Mechanically Mediated Microwave Frequency Conversion in the Quantum Regime.

Astrocyte NMDA receptors' activity sustains neuronal survival through a Cdk5-Nrf2 pathway.

Successful reversal of propionic acidaemia associated cardiomyopathy: evidence for low myocardial coenzyme Q10 status and secondary mitochondrial dysfunction as an underlying pathophysiological mechanism.

Mitochondrial dysfunction in the skeletal muscle of a mouse model of Rett syndrome (RTT): implications for the disease phenotype.

Effect of Coenzyme Q10 supplementation on mitochondrial electron transport chain activity and mitochondrial oxidative stress in Coenzyme Q10 deficient human neuronal cells.

Treatment of Fatty Acid Oxidation Disorders Today: Emerging Personalized Treatment Strategies.

International Guideline on the Diagnosis, Treatment, and Monitoring of Long-Chain Fatty Acid Oxidation Disorders (LC-FAOD).

L-Carnitine Prevents Lipopolysaccharide-Induced Inflammatory Response, Microglia Activation and Neuronal Damage in Cerebral Cortex, as Well as Neuromotor Development Delay in Neonatal Glutaryl-CoA Dehydrogenase Deficient (Gcdh-/-) Mice.

Longitudinal Assessment of Cognitive Development in 23 Patients With Mucopolysaccharidosis (MPS) Type II: Results of up to 14 Years of Follow-Up.

Distinct Urea Cycle Dysfunction Profiles Differentiate Acute Metabolic Decompensation in TMEM70 and MT-ATP6-Related Mitochondrial ATP Synthase Defects.

Sarcopenia in Pediatric Intoxication Type Inborn Errors of Metabolism: A Frequent and Underrecognized Condition.

Limbic-Predominant Cortical Magnetic Susceptibility Alterations Are Associated With Glycemic Dysregulation and Cognitive Performance in Type 2 Diabetes.

Efficacy of Dapagliflozin vs. Saroglitazar on Hepatic Steatosis and Fibrosis in Patients of Type 2 Diabetes Mellitus and Metabolic-Associated Steatotic Liver Disease (MASLD).

A 48-Month, Prospective, Multicenter, Natural History Study of Choroideremia.

Therapeutic and chemopreventive potential of melatonin in liver diseases: targeting oxidative stress and inflammatory signaling.

Mitochondrial permeability transition in skeletal muscle phenocopies muscle alterations seen in cancer cachexia and other wasting conditions.

A cardiometabolic risk phenotype index integrating insulin resistance, systemic inflammation, and psychological burden for mortality risk stratification in metabolic dysfunction-associated steatotic liver disease: an exploratory longitudinal analysis of a nationally representative cohort.

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Mitochondrial damage: an important feature in a number of inborn errors of metabolism?

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