Ethylmalonic Acid Induces Permeability Transition in Isolated Brain Mitochondria

The mitochondrial permeability transition pore: a mystery solved?

(2013) Paolo Bernardi   Frontiers in Physiology

Mitochondria as a Source of Reactive Oxygen and Nitrogen Species: From Molecular Mechanisms to Human Health

(2012) Tiago R. Figueira et al.   ANTIOXIDANTS & REDOX SIGNALING

The permeability transition pore as a Ca2+ release channel: New answers to an old question

(2012) Paolo Bernardi et al.   CELL CALCIUM

Prerequisites for ubiquinone analogs to prevent mitochondrial permeability transition-induced cell death

(2012) Julie Belliere et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Mitochondria as sensors and regulators of calcium signalling

(2012) Rosario Rizzuto et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Ethylmalonic acid modulates Na+, K+-ATPase activity and mRNA levels in rat cerebral cortex

(2012) Patrícia Fernanda Schuck et al.   SYNAPSE

Mitochondrial permeability transition in Ca2+-dependent apoptosis and necrosis

(2011) Andrea Rasola et al.   CELL CALCIUM

Morphologic evidence of diffuse vascular damage in human and in the experimental model of ethylmalonic encephalopathy

(2011) Carla Giordano et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Ethylmalonic acid impairs brain mitochondrial succinate and malate transport

(2011) Alexandre Umpierrez Amaral et al.   MOLECULAR GENETICS AND METABOLISM

Vulnerability to Oxidative Stress In Vitro in Pathophysiology of Mitochondrial Short-Chain Acyl-CoA Dehydrogenase Deficiency: Response to Antioxidants

(2011) Zarazuela Zolkipli et al.   PLoS One

The molecular identity of the mitochondrial Ca2+ sequestration system

(2010) Anatoly A. Starkov   FEBS Journal

Antioxidant dysfunction: potential risk for neurotoxicity in ethylmalonic aciduria

(2010) Christina B. Pedersen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Inhibitory Effects of Adenine Nucleotides on Brain Mitochondrial Permeability Transition

(2010) Ângela Saito et al.   NEUROCHEMICAL RESEARCH

Tissue-, substrate-, and site-specific characteristics of mitochondrial reactive oxygen species generation

(2009) Erich B. Tahara et al.   FREE RADICAL BIOLOGY AND MEDICINE

Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy

(2009) Valeria Tiranti et al.   NATURE MEDICINE

Promotion of Lipid and Protein Oxidative Damage in Rat Brain by Ethylmalonic Acid

(2009) Patrícia Fernanda Schuck et al.   NEUROCHEMICAL RESEARCH

Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit

(2008) Patrícia Fernanda Schuck et al.   BEHAVIOURAL BRAIN RESEARCH

The ACADS gene variation spectrum in 114 patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency is dominated by missense variations leading to protein misfolding at the cellular level

(2008) Christina B. Pedersen et al.   HUMAN GENETICS

Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial succinate uptake

(2008) S. R. Mirandola et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Quinolinate-induced Rat Striatal Excitotoxicity Impairs Endoplasmic Reticulum Ca2+-ATPase Function

(2008) Anna M. A. P. Fernandes et al.   NEUROCHEMICAL RESEARCH

Short-chain acyl-CoA dehydrogenase gene mutation (c.319C>T) presents with clinical heterogeneity and is candidate founder mutation in individuals of Ashkenazi Jewish origin

(2007) Ingrid Tein et al.   MOLECULAR GENETICS AND METABOLISM