(Q38515148)

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Disturbance of redox homeostasis as a contributing underlying pathomechanism of brain and liver alterations in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.

scientific article published on 04 June 2015

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scholarly article

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16 February 2020

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Europe PubMed Central

Disturbance of redox homeostasis as a contributing underlying pathomechanism of brain and liver alterations in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (English)

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26041581%20AND%20SRC:MED&resulttype=core&format=json

16 February 2020

3-hydroxy-3-methylglutaryl-CoA lyase deficiency

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Guilhian Leipnitz

1

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16 February 2020

3

object named as

Moacir Wajner

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16 February 2020

author name string

Carmen Regla Vargas

2

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16 February 2020

publication date

4 June 2015

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16 February 2020

Journal of Inherited Metabolic Disease

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16 February 2020

38

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16 February 2020

6

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16 February 2020

1021-1028

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16 February 2020

https://scigraph.springernature.com/pub.10.1007/s10545-015-9863-3

0 references

Mutations underlying 3-hydroxy-3-methylglutaryl CoA lyase deficiency in the Saudi population

1 reference

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Oxidative stress in neurodegeneration: cause or consequence?

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NADPH oxidase- and mitochondria-derived reactive oxygen species in proinflammatory microglial activation: a bipartisan affair?

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Nitric oxide and neuronal death

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Acetylcarnitine and cellular stress response: roles in nutritional redox homeostasis and regulation of longevity genes

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Developmental neuropathology of environmental agents.

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Redox homeostasis is compromised in vivo by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in rat cerebral cortex and liver.

1 reference

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In vivo intracerebral administration of L-2-hydroxyglutaric acid provokes oxidative stress and histopathological alterations in striatum and cerebellum of adolescent rats.

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In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: a potential pathophysiological mechanism of striatal damage in this diso

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Oxidative damage and the Nrf2-ARE pathway in neurodegenerative diseases

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A liver-specific defect of Acyl-CoA degradation produces hyperammonemia, hypoglycemia and a distinct hepatic Acyl-CoA pattern

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Urinary biomarkers of oxidative damage in Maple syrup urine disease: the L-carnitine role.

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'Liver let die': oxidative DNA damage and hepatotropic viruses

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Reactive oxygen and mechanisms of inflammatory liver injury: Present concepts

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Dysregulation of glutathione homeostasis in neurodegenerative diseases

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21 January 2018

Redefining oxidative stress

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Microglia, major player in the brain inflammation: their roles in the pathogenesis of Parkinson's disease

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Identifying, by first-principles simulations, Cu[amyloid-β] species making Fenton-type reactions in Alzheimer's disease

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21 January 2018

Evidence that 3-hydroxy-3-methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex.

1 reference

https://api.crossref.org/works/10.1007%2FS10545-015-9863-3

21 January 2018

Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats.

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21 January 2018

Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver.

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21 January 2018

Brain development and the immune system: an introduction to inflammatory and infectious diseases of the child's brain

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21 January 2018

Redox control of liver function in health and disease

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21 January 2018

Ten novel HMGCL mutations in 24 patients of different origin with 3-hydroxy-3-methyl-glutaric aciduria

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21 January 2018

Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect

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21 January 2018

Proteins in human brain cortex are modified by oxidation, glycoxidation, and lipoxidation. Effects of Alzheimer disease and identification of lipoxidation targets.

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21 January 2018

Oligomeric amyloid β induces IL-1β processing via production of ROS: implication in Alzheimer's disease

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21 January 2018

Oxidation of DNA, proteins and lipids by DOPA, protein-bound DOPA, and related catechol(amine)s

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21 January 2018

Molecular genetics of HMG-CoA lyase deficiency

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21 January 2018

Differential HMG-CoA lyase expression in human tissues provides clues about 3-hydroxy-3-methylglutaric aciduria

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21 January 2018

Analysis of aberrant splicing and nonsense-mediated decay of the stop codon mutations c.109G>T and c.504_505delCT in 7 patients with HMG-CoA lyase deficiency

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21 January 2018

3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: initial presentation in a young adult

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Biochemical reactivity of melatonin with reactive oxygen and nitrogen species: a review of the evidence

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L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders

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Fenton chemistry and oxidative stress mediate the toxicity of the beta-amyloid peptide in a Drosophila model of Alzheimer's disease

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Increased neuronal glucose-6-phosphate dehydrogenase and sulfhydryl levels indicate reductive compensation to oxidative stress in Alzheimer disease.

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A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency

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Oxidative stress and neurotoxicity

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

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ROS function in redox signaling and oxidative stress

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1 reference

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1 reference

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inferred from PubMed ID database lookup

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1 reference

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inferred from PubMed ID database lookup

An assessment of oxidative damage to proteins, lipids, and DNA in brain from patients with Alzheimer's disease.

1 reference

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MR imaging and proton spectroscopy in 3-hydroxy-3-methylglutaryl coenzyme A lyase deficiency.

1 reference

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1 reference

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