Bianca Seminotti Selected Research

Glutaric Acidemia I

1/2022	Increased susceptibility to quinolinic acid-induced seizures and long-term changes in brain oscillations in an animal model of glutaric acidemia type I.
11/2019	Pathogenesis of brain damage in glutaric acidemia type I: Lessons from the genetic mice model.
10/2019	Acute lysine overload provokes marked striatum injury involving oxidative stress signaling pathways in glutaryl-CoA dehydrogenase deficient mice.
1/2019	Long Lasting High Lysine Diet Aggravates White Matter Injury in Glutaryl-CoA Dehydrogenase Deficient (Gcdh-/-) Mice.
1/2018	Induction of Neuroinflammatory Response and Histopathological Alterations Caused by Quinolinic Acid Administration in the Striatum of Glutaryl-CoA Dehydrogenase Deficient Mice.
11/2016	Oxidative Stress, Disrupted Energy Metabolism, and Altered Signaling Pathways in Glutaryl-CoA Dehydrogenase Knockout Mice: Potential Implications of Quinolinic Acid Toxicity in the Neuropathology of Glutaric Acidemia Type I.
1/2014	Increased glutamate receptor and transporter expression in the cerebral cortex and striatum of gcdh-/- mice: possible implications for the neuropathology of glutaric acidemia type I.
9/2012	Marked reduction of Na(+), K(+)-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice.
6/2010	Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I.

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Bianca Seminotti Research Topics

Disease

9	Glutaric Acidemia I 01/2022 - 06/2010
4	Atrophy 01/2021 - 03/2010
3	Mitochondrial Diseases (Mitochondrial Disease) 10/2021 - 01/2017
3	Nonketotic Hyperglycinemia 01/2018 - 08/2011
3	Brain Injuries (Brain Injury) 09/2015 - 11/2012
3	Inborn Genetic Diseases (Disease, Hereditary) 06/2013 - 11/2009
2	Brain Diseases (Brain Disorder) 08/2022 - 12/2020
2	Phenylketonurias (Phenylketonuria) 06/2021 - 03/2010
2	Ethylmalonic encephalopathy 01/2021 - 01/2019
2	Gliosis 10/2019 - 01/2018
2	2-Hydroxyglutaricaciduria 01/2018 - 06/2015
2	Hyperlysinemias (Hyperlysinemia) 02/2011 - 06/2009
2	isovaleric Acidemia 03/2009 - 08/2008
1	Infections 01/2022
1	Seizures (Absence Seizure) 01/2022
1	Liver Diseases (Liver Disease) 10/2021
1	Inflammation (Inflammations) 12/2020
1	Alzheimer Disease (Alzheimer's Disease) 11/2020
1	Argininosuccinic Aciduria 02/2020
1	Reticular dysgenesis 10/2019
1	Demyelinating Diseases (Demyelinating Disease) 10/2019
1	Hypermethioninemia 04/2019
1	VLCAD deficiency 01/2019
1	Hyperammonemia 12/2012
1	Brain Edema (Cerebral Edema) 11/2012
1	Propionic Acidemia 07/2011
1	Methylmalonic acidemia 07/2011
1	Peroxisomal Disorders (Peroxisomal Disorder) 03/2011
1	Neurologic Manifestations (Neurological Manifestations) 02/2011
1	Refsum Disease (Refsum's Disease) 08/2010
1	Glioma (Gliomas) 06/2010
1	Intellectual Disability (Idiocy) 03/2010
1	Microcephaly 03/2010
1	Neurodegenerative Diseases (Neurodegenerative Disease) 06/2009
1	3-Methylglutaconic Aciduria 03/2008

Drug/Important Bio-Agent (IBA)

12	Glutaryl-CoA DehydrogenaseIBA 08/2022 - 09/2012
9	AcidsIBA 01/2022 - 03/2008
8	Lysine (L-Lysine)FDA Link 01/2022 - 06/2009
4	Biological ProductsIBA 10/2021 - 02/2011
4	EnzymesIBA 11/2019 - 06/2009
3	Tryptophan (L-Tryptophan)FDA Link 01/2022 - 01/2019
3	HydroxylysineIBA 01/2022 - 01/2019
3	Glycine (Aminoacetic Acid)FDA LinkGeneric 01/2018 - 08/2011
3	Adenosine Triphosphatases (ATPase)IBA 11/2012 - 06/2009
2	Quinolinic AcidIBA 01/2022 - 11/2016
2	S-Adenosylmethionine (Ademetionine)IBA 10/2021 - 04/2019
2	Phenylalanine (L-Phenylalanine)FDA Link 06/2021 - 03/2010
2	Amino Acids FDA Link 01/2019 - 02/2011
2	Proteins (Proteins, Gene)FDA Link 01/2019 - 01/2018
2	AntioxidantsIBA 01/2017 - 06/2015
2	Reactive Oxygen Species (Oxygen Radicals)IBA 06/2015 - 03/2011
2	Glutathione (Reduced Glutathione)IBA 12/2012 - 06/2010
2	OrnithineIBA 12/2012 - 11/2009
2	homocitrullineIBA 12/2012 - 11/2009
2	HHH syndromeIBA 12/2012 - 11/2009
2	isovaleric acidIBA 03/2009 - 08/2008
1	KynurenineIBA 01/2022
1	Adenosine KinaseIBA 10/2021
1	Glycine N-Methyltransferase (Glycine Sarcosine N-Methyltransferase)IBA 10/2021
1	AdenosylhomocysteinaseIBA 10/2021
1	Mitochondrial DNA (mtDNA)IBA 10/2021
1	Phenylalanine Hydroxylase (Phenylalanine 4 Monooxygenase)IBA 06/2021
1	ethylmalonic acid (ethylmalonate)IBA 01/2021
1	LipopolysaccharidesIBA 12/2020
1	Amyloid (Amyloid Fibrils)IBA 11/2020
1	Argininosuccinic AcidIBA 02/2020
1	Free Radical ScavengersIBA 02/2020
1	adenylate kinase 2IBA 10/2019
1	SulfurIBA 01/2019
1	Fatty Acids (Saturated Fatty Acids)IBA 01/2019
1	Long-Chain Acyl-CoA Dehydrogenase (Long-Chain-Acyl-Coenzyme A Dehydrogenase)IBA 01/2019
1	molybdopterin (molybdenum cofactor)IBA 01/2019
1	Vitamin K 3 (Menadione)FDA Link 01/2017
1	Ketoglutaric Acids (Ketoglutaric Acid)IBA 06/2015
1	Riboflavin (Vitamin B2)FDA LinkGeneric 06/2015
1	Carnitine (L-Carnitine)FDA LinkGeneric 06/2015
1	Glutamate Receptors (Glutamate Receptor)IBA 01/2014
1	3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA)IBA 06/2013
1	LyasesIBA 06/2013
1	Nitric Oxide (Nitrogen Monoxide)FDA Link 12/2012
1	CatalaseIBA 12/2012
1	AmmoniaIBA 12/2012
1	UreaseIBA 12/2012
1	Glutathione PeroxidaseIBA 12/2012
1	propionic acid (potassium propionate)IBA 07/2011
1	Methylmalonic AcidIBA 07/2011
1	pristanic acidIBA 03/2011
1	Phytanic AcidIBA 08/2010
1	Thiobarbituric Acid Reactive SubstancesIBA 06/2010
1	Adenosine Triphosphate (ATP)IBA 06/2009
1	Acetic Acid (Vinegar)FDA LinkGeneric 06/2009
1	Glucose (Dextrose)FDA LinkGeneric 06/2009
1	Creatine Kinase (Creatine Phosphokinase)IBA 06/2009
1	Isovaleryl-CoA Dehydrogenase (Isovaleryl-Coenzyme A Dehydrogenase)IBA 03/2009

Therapy/Procedure

2	Therapeutics 02/2020 - 06/2015
1	Protein-Restricted Diet (Diet, Protein Restricted) 06/2015