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Inhibition of Endothelial Notch Signaling Impairs Fatty Acid Transport and Leads to Metabolic and Vascular Remodeling of the Adult Heart

Circulation (New York, N.Y.), 2018-06, Vol.137 (24), p.2592-2608

2018

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T cell Activation Is Driven by an ADP-Dependent Glucokinase Linking Enhanced Glycolysis with Mitochondrial Reactive Oxygen Species Generation

Cell reports (Cambridge), 2012-11, Vol.2 (5), p.1300-1315

2012

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DHTKD1 Mutations Cause 2-Aminoadipic and 2-Oxoadipic Aciduria

American journal of human genetics, 2012-12, Vol.91 (6), p.1082-1087

2012

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QDPR homologues in Danio rerio regulate melanin synthesis, early gliogenesis, and glutamine homeostasis

PloS one, 2019-04, Vol.14 (4), p.e0215162-e0215162

2019

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The HMGB1 protein induces a metabolic type of tumour cell death by blocking aerobic respiration

Nature communications, 2016-03, Vol.7 (1), p.10764-10764, Article 10764

2016

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Current concepts in organic acidurias: understanding intra- and extracerebral disease manifestation

Journal of inherited metabolic disease, 2013-07, Vol.36 (4), p.635-644

2013

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Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins

Biochemical journal, 2006-08, Vol.398 (1), p.107-112

2006

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Renal dysfunction in methylmalonic acidurias: review for the pediatric nephrologist

Pediatric nephrology (Berlin, West), 2013-02, Vol.28 (2), p.227-235

2013

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Crystal structure and interaction studies of human DHTKD1 provide insight into a mitochondrial megacomplex in lysine catabolism

IUCrJ, 2020-07, Vol.7 (Pt 4), p.693-706

2020

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Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I

Journal of inherited metabolic disease, 2015-03, Vol.38 (2), p.265-272

2015

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Glutaric aciduria type I and methylmalonic aciduria: Simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood–brain barrier and the choroid plexus

Biochimica et biophysica acta, 2010-06, Vol.1802 (6), p.552-560

2010

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Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency

Journal of neurochemistry, 2006-05, Vol.97 (3), p.899-910

2006

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Mitochondrial reactive oxygen species control T cell activation by regulating IL-2 and IL-4 expression: mechanism of ciprofloxacin-mediated immunosuppression

The Journal of immunology (1950), 2010-05, Vol.184 (9), p.4827-4841

2010

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Iron Regulatory Proteins Secure Mitochondrial Iron Sufficiency and Function

Cell metabolism, 2010-08, Vol.12 (2), p.194-201

2010

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Therapeutic modulation of cerebral L-lysine metabolism in a mouse model for glutaric aciduria type I

Brain (London, England : 1878), 2011, Vol.134 (Pt 1), p.157-170

2011

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Xanthohumol-induced transient superoxide anion radical formation triggers cancer cells into apoptosis via a mitochondria-mediated mechanism

The FASEB journal, 2010-08, Vol.24 (8), p.2938-2950

2010

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Impairment of astrocytic glutaminolysis in glutaric aciduria type I

Journal of inherited metabolic disease, 2018, Vol.41 (1), p.91-99

2018

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Cellular citrate levels establish a regulatory link between energy metabolism and the hepatic iron hormone hepcidin

Journal of molecular medicine (Berlin, Germany), 2017-08, Vol.95 (8), p.851-860

2017

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Multifactorial modulation of susceptibility to l-lysine in an animal model of glutaric aciduria type I

Biochimica et biophysica acta, 2015-05, Vol.1852 (5), p.768-777

2015

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The lipid-droplet-associated protein ABHD5 protects the heart through proteolysis of HDAC4

Nature metabolism, 2019-11, Vol.1 (11), p.1157-1167

2019

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Severe dysfunction of respiratory chain and cholesterol metabolism in Atp7b −/− mice as a model for Wilson disease

Biochimica et biophysica acta, 2011-12, Vol.1812 (12), p.1607-1615

2011

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Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A

The Journal of biological chemistry, 2005-06, Vol.280 (23), p.21830-21836

2005

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Bioenergetics in Glutaryl-Coenzyme A Dehydrogenase Deficiency

The Journal of biological chemistry, 2005-06, Vol.280 (23), p.21830-21836

2005

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Inhibition of Endothelial Notch Signaling Impairs Fatty Acid Transport and Leads to Metabolic and Vascular Remodeling of the Adult Heart

T cell Activation Is Driven by an ADP-Dependent Glucokinase Linking Enhanced Glycolysis with Mitochondrial Reactive Oxygen Species Generation

DHTKD1 Mutations Cause 2-Aminoadipic and 2-Oxoadipic Aciduria

QDPR homologues in Danio rerio regulate melanin synthesis, early gliogenesis, and glutamine homeostasis

The HMGB1 protein induces a metabolic type of tumour cell death by blocking aerobic respiration

Current concepts in organic acidurias: understanding intra- and extracerebral disease manifestation

Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins

Renal dysfunction in methylmalonic acidurias: review for the pediatric nephrologist

Crystal structure and interaction studies of human DHTKD1 provide insight into a mitochondrial megacomplex in lysine catabolism

Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I

Glutaric aciduria type I and methylmalonic aciduria: Simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood–brain barrier and the choroid plexus

Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency

A proteolytic fragment of histone deacetylase 4 protects the heart from failure by regulating the hexosamine biosynthetic pathway

Mitochondrial reactive oxygen species control T cell activation by regulating IL-2 and IL-4 expression: mechanism of ciprofloxacin-mediated immunosuppression

Iron Regulatory Proteins Secure Mitochondrial Iron Sufficiency and Function

Therapeutic modulation of cerebral L-lysine metabolism in a mouse model for glutaric aciduria type I

Xanthohumol-induced transient superoxide anion radical formation triggers cancer cells into apoptosis via a mitochondria-mediated mechanism

Impairment of astrocytic glutaminolysis in glutaric aciduria type I

Cellular citrate levels establish a regulatory link between energy metabolism and the hepatic iron hormone hepcidin

Multifactorial modulation of susceptibility to l-lysine in an animal model of glutaric aciduria type I

The lipid-droplet-associated protein ABHD5 protects the heart through proteolysis of HDAC4

Severe dysfunction of respiratory chain and cholesterol metabolism in Atp7b −/− mice as a model for Wilson disease

Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A

Bioenergetics in Glutaryl-Coenzyme A Dehydrogenase Deficiency

A novel autosomal recessive TERT T1129P mutation in a dyskeratosis congenita family leads to cellular senescence and loss of CD34+ hematopoietic stem cells not reversible by mTOR-inhibition

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