Cited by 166Amyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatmentsMitochondrial Function and Pathology

Cell‐permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis

Petri, Susanne, Kiaei, Mahmoud, Damiano, Maria, Hiller, Andrew, Wille, Elizabeth, Manfredi, Giovanni, Calingasan, Noel Y., Szeto, Hazel H. +1 more·2006·Journal of Neurochemistry

Abstract

Reactive oxygen species (ROS) play a major role in the pathogenesis of neurodegenerative diseases. They are important contributors to necrotic and apoptotic cell death. A major proportion of cellular ROS is generated at the inner mitochondrial membrane by the respiratory chain. In the present study, we investigated a novel peptide antioxidant (SS‐31) targeted to the inner mitochondrial membrane for its therapeutic effects both in vitro and in vivo in the G93A mouse model of amyotrophic lateral sclerosis (ALS). SS‐31 protected against cell death induced by hydrogen peroxide in vitro in neuronal cells stably transfected with either wild‐type or mutant Cu/Zn superoxide dismutase (SOD1). Daily intraperitoneal injections of SS‐31 (5 mg/kg), starting at 30 days of age, led to a significant improvement in survival and motor performance. In comparison with vehicle‐treated G93A mice, SS‐31‐treated mice showed a decreased cell loss and a decrease in immunostaining for markers of oxidative stress in the lumbar spinal cord. This further enhances the concept that pharmacological modification of oxidative stress is a therapeutic option for the treatment of ALS.

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Crossref · 10.1111/j.1471-4159.2006.04018.x

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  • SS-31 mediated significant neuroprotection against cell death induced by hydrogen peroxide in SOD1 neuronal cells.

    “We found SS-31 mediated significant neuroprotection against cell death induced by hydrogen peroxide in SOD1 neuronal cells.”

    From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [180]

  • Daily intraperitoneal injections of SS-31 (5 mg/kg), starting at 30 days of age, led to a significant improvement in survival and motor performance in the G93A transgenic mouse model of ALS.

    “Further, daily intraperitoneal injections of SS-31 (5 mg/kg), starting at 30 days of age, led to a significant improvement in survival and motor performance in the G93A transgenic mouse model of ALS.”

    From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [180]

  • Such peptides reduce intracellular ROS and cell death in neuronal and pancreatic islet cells and reduce skeletal myocyte H2O2 production and preserve insulin sensitivity in rats fed a high-fat diet.

    “Such peptides reduce intracellular ROS and cell death in neuronal (256) and pancreatic islet cells (309) and recently were reported to reduce skeletal myocyte H2O2 production and to preserve insulin sensitivity in rats fed a high-fat diet (14).”

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Authors
Petri, S., Kiaei, M., Damiano, M., Hiller, A., Wille, E., Manfredi, G., Calingasan, N. Y., Szeto, H. H., & Beal, M. F.
Affiliations
Cornell University (US)
Venue
Journal of Neurochemistry
Publisher
Wiley
Published
2006-07-10
Volume
98
Issue
4
Pages
1141-1148
ISSN
  • 0022-3042 (ISSN-L)
  • 1471-4159
OpenAlex
W2036154524
Cited by
166
References
46
Citations by year
2026: 1 · 2025: 3 · 2024: 12 · 2023: 4 · 2022: 11 · 2021: 12 · 2020: 3 · 2019: 9 · 2018: 10 · 2017: 4 · 2016: 10 · 2015: 10 · 2014: 12 · 2013: 6 · 2012: 10
FWCI
6.41
Type
journal article
Language
en
Open access
closed
Locations
  • Journal of Neurochemistry (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • ALS Association
  • Muscular Dystrophy Association
  • Deutsche Forschungsgemeinschaft
  • U.S. Public Health Service (NINDS 48295, NIDA08924)
  • National Institute of Neurological Disorders and Stroke
Topics
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Keywords
amyotrophic lateral sclerosis, SS-31, reactive oxygen species, oxidative stress, Cu/Zn superoxide dismutase, inner mitochondrial membrane, hydrogen peroxide-induced cell death, neuroprotection, motor performance, lumbar spinal cord
MeSH terms
Superoxide Dismutase-1, Aging, Amyotrophic Lateral Sclerosis, Animals, Antioxidants, Body Weight, Cell Count, Cell Membrane Permeability, Cells, Cultured, Disease Models, Animal, Mice, Transgenic, Peptides, Psychomotor Performance, Spinal Cord, Superoxide Dismutase, Reactive Oxygen Species, Mice

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