Cited by 441Amyotrophic Lateral Sclerosis ResearchSpinal Cord Injury ResearchNeurogenetic and Muscular Disorders Research

Focal transplantation–based astrocyte replacement is neuroprotective in a model of motor neuron disease

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Lepore, Angelo C, Rauck, Britta, Dejea, Christine, Pardo, Andrea C, Rao, Mahendra S, Rothstein, Jeffrey D, Maragakis, Nicholas J·2008·Nature Neuroscience

Abstract

Cellular abnormalities in amyotrophic lateral sclerosis (ALS) are not limited to motor neurons. Astrocyte dysfunction also occurs in human ALS and transgenic rodents expressing mutant human SOD1 protein (SOD1(G93A)). Here we investigated focal enrichment of normal astrocytes using transplantation of lineage-restricted astrocyte precursors, called glial-restricted precursors (GRPs). We transplanted GRPs around cervical spinal cord respiratory motor neuron pools, the principal cells whose dysfunction precipitates death in ALS. GRPs survived in diseased tissue, differentiated efficiently into astrocytes and reduced microgliosis in the cervical spinal cords of SOD1(G93A) rats. GRPs also extended survival and disease duration, attenuated motor neuron loss and slowed declines in forelimb motor and respiratory physiological functions. Neuroprotection was mediated in part by the primary astrocyte glutamate transporter GLT1. These findings indicate the feasibility and efficacy of transplantation-based astrocyte replacement and show that targeted multisegmental cell delivery to the cervical spinal cord is a promising therapeutic strategy for slowing focal motor neuron loss associated with ALS.

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Authors
Lepore, A. C., Rauck, B., Dejea, C., Pardo, A. C., Rao, M. S., Rothstein, J. D., & Maragakis, N. J.
Affiliations
Johns Hopkins University (US)
Corresponding author
Maragakis, Nicholas J
Venue
Nature Neuroscience
Published
2008-10-19
Volume
11
Issue
11
Pages
1294-1301
ISSN
  • 1097-6256 (ISSN-L)
  • 1546-1726
OpenAlex
W2121396070
Cited by
441
References
51
Citations by year
2026: 7 · 2025: 9 · 2024: 16 · 2023: 34 · 2022: 22 · 2021: 25 · 2020: 23 · 2019: 19 · 2018: 22 · 2017: 15 · 2016: 20 · 2015: 28 · 2014: 34 · 2013: 21 · 2012: 39
FWCI
22.68
Type
journal article
Language
en
Open access
Locations
  • Nature Neuroscience (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
Funders
National Institute of Neurological Disorders and Stroke (R01 NS41680, R01NS041680, R01-NS33958, F32NS059155, R01NS033958)
Topics
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Spinal Cord Injury Research
  • Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
Keywords
amyotrophic lateral sclerosis, astrocyte transplantation, SOD1 G93A, superoxide dismutase 1, neuroprotection, motor neuron degeneration, microglial activation, cervical spinal cord, respiratory motor neurons, astrocyte dysfunction
MeSH terms
Action Potentials, Animals, Astrocytes, Calcium-Binding Proteins, Cell Differentiation, Cell Movement, Cell Survival, Disease Models, Animal, Glial Fibrillary Acidic Protein, Hindlimb, Humans, Microfilament Proteins, Neural Conduction, Phrenic Nerve, Spinal Cord, Stem Cells, Superoxide Dismutase, Motor Neuron Disease, Cell Transplantation, Muscle, Skeletal, Excitatory Amino Acid Transporter 2, Animals, Genetically Modified, Green Fluorescent Proteins, Rats

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