The DOI matches its Crossref record; 22 of the 22 references it cites were found in a registry or an index.
A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis
Open accessAbstract
Background: The cause of neuronal death in amyotrophic lateral sclerosis (ALS) is uncertain but mitochondrial dysfunction may play an important role. Ketones promote mitochondrial energy production and membrane stabilization. Results: SOD1-G93A transgenic ALS mice were fed a ketogenic diet (KD) based on known formulations for humans. Motor performance, longevity, and motor neuron counts were measured in treated and disease controls. Because mitochondrial dysfunction plays a central role in neuronal cell death in ALS, we also studied the effect that the principal ketone body, D-β-3 hydroxybutyrate (DBH), has on mitochondrial ATP generation and neuroprotection. Blood ketones were > 3.5 times higher in KD fed animals compared to controls. KD fed mice lost 50% of baseline motor performance 25 days later than disease controls. KD animals weighed 4.6 g more than disease control animals at study endpoint; the interaction between diet and change in weight was significant (p = 0.047). In spinal cord sections obtained at the study endpoint, there were more motor neurons in KD fed animals (p = 0.030). DBH prevented rotenone mediated inhibition of mitochondrial complex I but not malonate inhibition of complex II. Rotenone neurotoxicity in SMI-32 immunopositive motor neurons was also inhibited by DBH. Conclusion: This is the first study showing that diet, specifically a KD, alters the progression of the clinical and biological manifestations of the G93A SOD1 transgenic mouse model of ALS. These effects may be due to the ability of ketone bodies to promote ATP synthesis and bypass inhibition of complex I in the mitochondrial respiratory chain.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
Rosen, D. R., Siddique, T., Patterson, D., Figlewicz, D. A., Sapp, P., Hentati, A., Donaldson, D., Goto, J., O'Regan, J. P., Deng, H.-X., et al. (1993) · Nature · doi:10.1038/362059a0 · Cited by 7,062
Rowland, L. P., & Shneider, N. A. (2001) · New England Journal of Medicine · doi:10.1056/nejm200105313442207 · Cited by 1,812
Veech, R. L. (2004) · Prostaglandins, Leukotrienes and Essential Fatty Acids · doi:10.1016/j.plefa.2003.09.007 · Cited by 755
Mattiazzi, M., D'Aurelio, M., Gajewski, C. D., Martushova, K., Kiaei, M., Beal, M. F., & Manfredi, G. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m203065200 · Cited by 616
d -β-Hydroxybutyrate protects neurons in models of Alzheimer's and Parkinson's disease
Kashiwaya, Y., Takeshima, T., Mori, N., Nakashima, K., Clarke, K., & Veech, R. L. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.97.10.5440 · Cited by 535
Pasinelli, P., Belford, M. E., Lennon, N., Bacskai, B. J., Hyman, B. T., Trotti, D., & Brown, R. H. (2004) · Neuron · doi:10.1016/j.neuron.2004.06.021 · Cited by 506
D-β-Hydroxybutyrate rescues mitochondrial respiration and mitigates features of Parkinson disease
Tieu, K., Perier, C., Caspersen, C., Teismann, P., Wu, D.-C., Yan, S.-D., Naini, A., Vila, M., Jackson-Lewis, V., Ramasamy, R., et al. (2003) · Journal of Clinical Investigation · doi:10.1172/jci200318797 · Cited by 497
Insulin, ketone bodies, and mitochondrial energy transduction
Sato, K., Kashiwaya, Y., Keon, C. A., Tsuchiya, N., King, M. T., Radda, G. K., Chance, B., Clarke, K., & Veech, R. L. (1995) · The FASEB Journal · doi:10.1096/fasebj.9.8.7768357 · Cited by 454
Pitkanen, S., & Robinson, B. H. (1996) · Journal of Clinical Investigation · doi:10.1172/jci118798 · Cited by 388
Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients
Wiedemann, F. R., Manfredi, G., Mawrin, C., Beal, M. F., & Schon, E. A. (2002) · Journal of Neurochemistry · doi:10.1046/j.0022-3042.2001.00731.x · Cited by 359
Cited by
Ketone bodies as signaling metabolites
Newman, J. C., & Verdin, E. (2014) · Trends in Endocrinology & Metabolism · doi:10.1016/j.tem.2013.09.002 · Cited by 966
Beyond weight loss: a review of the therapeutic uses of very-low-carbohydrate (ketogenic) diets
Paoli, A., Rubini, A., Volek, J. S., & Grimaldi, K. A. (2013) · European Journal of Clinical Nutrition · doi:10.1038/ejcn.2013.116 · Cited by 885
Henderson, S. T., Vogel, J. L., Barr, L. J., Garvin, F., Jones, J. J., & Costantini, L. C. (2009) · Nutrition & Metabolism · doi:10.1186/1743-7075-6-31 · Cited by 548
The Ketogenic Diet as a Treatment Paradigm for Diverse Neurological Disorders
Stafstrom, C. E., & Rho, J. M. (2012) · Frontiers in Pharmacology · doi:10.3389/fphar.2012.00059 · Cited by 438
Metabolic Control of Vesicular Glutamate Transport and Release
Juge, N., Gray, J. A., Omote, H., Miyaji, T., Inoue, T., Hara, C., Uneyama, H., Edwards, R. H., Nicoll, R. A., & Moriyama, Y. (2010) · Neuron · doi:10.1016/j.neuron.2010.09.002 · Cited by 400
Metadata
- Authors
- Zhao, Z., Lange, D. J., Voustianiouk, A., MacGrogan, D., Ho, L., Suh, J., Humala, N., Thiyagarajan, M., Wang, J., & Pasinetti, G. M.
- Venue
- BMC Neuroscience
- Published
- 2006-04-03
- Volume
- 7
- Issue
- 1
- Pages
- 29-29
- ISSN
- 1471-2202 (ISSN-L)
- OpenAlex
- W2102247427
- Cited by
- 286
- References
- 22
- FWCI
- 5.02
- Type
- journal article
- Language
- en
- Open access
- gold
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
How to cite
Cite CoChat for the checks on this page, and the paper for its own findings.
- This page
- CoChat. (2026). Citation check: A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis. https://app.cochat.ai/papers/10.1186/1471-2202-7-29
- The paper
- Zhao, Z., Lange, D. J., Voustianiouk, A., MacGrogan, D., Ho, L., Suh, J., Humala, N., Thiyagarajan, M., Wang, J., & Pasinetti, G. M. (2006). A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis. BMC Neuroscience. https://doi.org/10.1186/1471-2202-7-29
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
