Butyrate Ameliorates Mitochondrial Respiratory Capacity of The Motor-Neuron-like Cell Line NSC34-G93A, a Cellular Model for ALS
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Mitochondrial defects in motor neurons are pathological hallmarks of ALS, a neuromuscular disease with no effective treatment. Studies have shown that butyrate, a natural gut-bacteria product, alleviates the disease progression of ALS mice overexpressing a human ALS-associated mutation, hSOD1G93A. In the current study, we examined the potential molecular mechanisms underlying the effect of butyrate on mitochondrial function in cultured motor-neuron-like NSC34 with overexpression of hSOD1G93A (NSC34-G93A). The live cell confocal imaging study demonstrated that 1mM butyrate in the culture medium improved the mitochondrial network with reduced fragmentation in NSC34-G93A cells. Seahorse analysis revealed that NSC34-G93A cells treated with butyrate showed an increase of ~5-fold in mitochondrial Spare Respiratory Capacity with elevated Maximal Respiration. The time-dependent changes in the mRNA level of PGC1α, a master regulator of mitochondrial biogenesis, revealed a burst induction with an early increase (~5-fold) at 4 h, a peak at 24 h (~19-fold), and maintenance at 48 h (8-fold) post-treatment. In line with the transcriptional induction of PGC1α, both the mRNA and protein levels of the key molecules (MTCO1, MTCO2, and COX4) related to the mitochondrial electron transport chain were increased following the butyrate treatment. Our data indicate that activation of the PGC1α signaling axis could be one of the molecular mechanisms underlying the beneficial effects of butyrate treatment in improving mitochondrial bioenergetics in NSC34-G93A cells.
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In motor neuron-like NSC34 cells overexpressing hSOD1G93A, butyrate improves mitochondrial bioenergetics by improving the mitochondrial network and upregulating transcription of PGC1α.
“Using motor neuron-like NSC34 cells with overexpression of hSOD1G93A, it was found that butyrate improved mitochondrial bioenergetics by improving mitochondrial network and upregulating the transcription of peroxisome proliferator-activated receptor-gamma coactivator-1α (PGC1α).348”
From Microbiota–gut–brain axis and its therapeutic applications in neurodegenerative diseases (2024) · cites this paper as [348]
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Self-citationLisinopril activates BI1 to reprogram lipid metabolism and restore autophagy in ALS
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- Authors
- Li, X., Dong, L., Li, A., Yi, J., Brotto, M., & Zhou, J.
- Affiliations
- The University of Texas at Arlington (US)
- Corresponding author
- Zhou, Jingsong
- Venue
- Biomolecules
- Publisher
- MDPI AG
- Published
- 2022-02-19
- Volume
- 12
- Issue
- 2
- Pages
- 333
- ISSN
- 2218-273X (ISSN-L)
- PMC
- PMC8869540
- OpenAlex
- W4213295538
- Cited by
- 33
- References
- 85
- Citations by year
- 2026: 9 · 2025: 6 · 2024: 9 · 2023: 7 · 2022: 2
- FWCI
- 3.16
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Biomolecules (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Institutes of Health (R01NS105621, R01 HL138570)
- United States Department of Defense (AL170061(W81XWH1810684))
- Amyotrophic Lateral Sclerosis Association (16-IIP-288)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Keywords
- butyrate, amyotrophic lateral sclerosis, mitochondrial respiratory capacity, motor neuron-like cells, SOD1 G93A mutation, PGC-1α, mitochondrial biogenesis, mitochondrial fragmentation, mitochondrial electron transport chain, gut microbiota metabolites, mitochondrial bioenergetics
- MeSH terms
- Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha, Amyotrophic Lateral Sclerosis, Animals, Butyrates, Cell Line, Disease Models, Animal, Mice, Transgenic, Mitochondria, Motor Neurons, RNA, Messenger, Superoxide Dismutase, Mice
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