ArticleNeurobiology of disease2011
Decreased glutathione accelerates neurological deficit and mitochondrial pathology in familial ALS-linked hSOD1(G93A) mice model.
Article in Neurobiology of disease, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
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Who cites it
41 citing papers in PubMed, 69 citations in OpenAlex.
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- Nicotinamide Adenine Dinucleotide Precursor Supplementation Modulates Neurite Complexity and Survival in Motor Neurons from Amyotrophic Lateral Sclerosis Models.Antioxidants & redox signaling · 2024Article
- Ferroptosis regulation through Nrf2 and implications for neurodegenerative diseases.Archives of toxicology · 2024Review
- Mitochondria: A Promising Convergent Target for the Treatment of Amyotrophic Lateral Sclerosis.Cells · 2024Review
- Honokiol alleviated neurodegeneration by reducing oxidative stress and improving mitochondrial function in mutant SOD1 cellular and mouse models of amyotrophic lateral sclerosis.Acta pharmaceutica Sinica. B · 2023Article
- NR1D1 downregulation in astrocytes induces a phenotype that is detrimental to cocultured motor neurons.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2022Article
- Activation of the Nrf2 Pathway as a Therapeutic Strategy for ALS Treatment.Molecules (Basel, Switzerland) · 2022Review
- Glutathione in the Nervous System as a Potential Therapeutic Target to Control the Development and Progression of Amyotrophic Lateral Sclerosis.Antioxidants (Basel, Switzerland) · 2021Review
- Overexpression of ferroptosis defense enzyme Gpx4 retards motor neuron disease of SOD1G93A mice.Scientific reports · 2021Article
- The Link between Oxidative Stress, Redox Status, Bioenergetics and Mitochondria in the Pathophysiology of ALS.International journal of molecular sciences · 2021Review
- Nicotinamide Riboside and Pterostilbene Cooperatively Delay Motor Neuron Failure in ALS SOD1Molecular neurobiology · 2021Article
- Altered expression of clock and clock-controlled genes in a hSOD1-linked amyotrophic lateral sclerosis mouse model.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2021Article
- Mitochondria Dysfunction in Frontotemporal Dementia/Amyotrophic Lateral Sclerosis: Lessons FromFrontiers in neuroscience · 2021Review
- Zingerone [4-(3-Methoxy-4-hydroxyphenyl)-butan-2] Attenuates Lipopolysaccharide-Induced Inflammation and Protects Rats from Sepsis Associated Multi Organ Damage.Molecules (Basel, Switzerland) · 2020Article
- Imaging Neurochemistry and Brain Structure Tracks Clinical Decline and Mechanisms of ALS in Patients.Frontiers in neurology · 2020Article
- Oxidative Stress in Amyotrophic Lateral Sclerosis: Pathophysiology and Opportunities for Pharmacological Intervention.Oxidative medicine and cellular longevity · 2020Review
- Human iPSC-derived astrocytes from ALS patients with mutated C9ORF72 show increased oxidative stress and neurotoxicity.EBioMedicine · 2019Article
- Impaired Pentose Phosphate Pathway in the Spinal Cord of the hSOD1Molecular neurobiology · 2019Article
- Cu/Zn-superoxide dismutase and wild-type like fALS SOD1 mutants produce cytotoxic quantities of HScientific reports · 2019Article
- Mitophagy links oxidative stress conditions and neurodegenerative diseases.Neural regeneration research · 2019Review
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3 authors at 1 institution in 1 country.
Funding
Abstract
Dominant mutations in Cu/Zn-superoxide dismutase (SOD1) cause familial forms of amyotrophic lateral sclerosis (ALS), a fatal disorder characterized by the progressive loss of motor neurons. To investigate the role of antioxidant defenses in ALS we used knockout mice for the glutamate-cysteine ligase modifier subunit (GCLM-/-), which have a 70-80% reduction in total glutathione. Although GCLM(-/-) mice are viable and fertile, the life span of GCLM(-/-)/hSOD1(G93A) mice decreased in 55% when compared to GCLM(+/+)/hSOD1(G93A) mice. Decreased life span in GCLM(-/-)/hSOD1(G93A) mice was associated to increased oxidative stress, aggravated mitochondrial pathology and increased association of hSOD1 with the mitochondria. Interestingly, when the GCLM(-/-) animals were mated with a different ALS-model which overexpress the experimental mutation hSOD1(H46R/H48Q), no effect was observed in survival of GCLM(-/-)/hSOD1(H46R/H48Q) mice; and little or no mitochondrial pathology was observed. Since a specific disease modifier, such as glutathione deficiency, may affect only certain hSOD1 mutants, these findings contribute to our understanding of the potential difference in the molecular pathways by which different hSOD1 mutants generate disease.
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