The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.
Complex I Defect in muscle from patients with Huntington's disease
Abstract
We found a variable defect of complex I of the mitochondrial respiratory chain, ranging in severity from 25% to 63% of control values, in muscle of patients with Huntington's disease (HD). The most severe defect was observed in the patient with the greatest expansion of CAG triplets. Muscle morphology showed myopathic changes such as moth‐eaten fibers, angulated fibers, increased subsarcolemmal oxidative activities, or an increased number of enlarged mitochondria with abnormal cristae. Multiple mitochondrial DNA deletions were found by polymerase chain reaction (PCR) analysis in muscle of the patient with the most severe defect of complex I. Our data further support the involvement of energetic defects and oxidative damage in muscle of patients with HD.
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MACDONALD, M. (1993) · Cell · doi:10.1016/0092-8674(93)90585-e · Cited by 8,366
Browne, S. E., Bowling, A. C., Macgarvey, U., Baik, M. J., Berger, S. C., Muquit, M. M. K., Bird, E. D., & Beal, M. F. (1997) · Annals of Neurology · doi:10.1002/ana.410410514 · Cited by 885
Mitochondrial defect in Huntington's disease caudate nucleus
Gu, M., Gash, M. T., Mann, V. M., Javoy‐Agid, F., Cooper, J. M., & Schapira, A. H. V. (1996) · Annals of Neurology · doi:10.1002/ana.410390317 · Cited by 758
Energy metabolism defects in Huntington's disease and effects of coenzyme Q10
Koroshetz, W. J., Jenkins, B. G., Rosen, B. R., & Beal, M. F. (1997) · Annals of Neurology · doi:10.1002/ana.410410206 · Cited by 405
Cited by
Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Zuccato, C., Valenza, M., & Cattaneo, E. (2010) · Physiological Reviews · doi:10.1152/physrev.00041.2009 · Cited by 848
Huntington’s Disease: Mechanisms of Pathogenesis and Therapeutic Strategies
Jimenez-Sanchez, M., Licitra, F., Underwood, B. R., & Rubinsztein, D. C. (2017) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a024240 · Cited by 507
Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse
Tabrizi, S. J., Workman, J., Hart, P. E., Mangiarini, L., Mahal, A., Bates, G., Cooper, J. M., & Schapira, A. H. V. (2000) · Annals of Neurology · doi:10.1002/1531-8249(200001)47:1<80::aid-ana13>3.0.co;2-k · Cited by 351
Schapira, A. H. V. (1999) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/s0005-2728(98)00164-9 · Cited by 310
Mitochondrial Approaches for Neuroprotection
Chaturvedi, R. K., & Beal, M. F. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.027 · Cited by 294
Metadata
- Authors
- Arenas, J., Campos, Y., Ribacoba, R., Martín, M. A., Rubio, J. C., Ablanedo, P., & Cabello, A.
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 1998-03
- Volume
- 43
- Issue
- 3
- Pages
- 397-400
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W2133902481
- Cited by
- 173
- References
- 4
- FWCI
- 2.48
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Complex I Defect in muscle from patients with Huntington's disease. https://app.cochat.ai/papers/10.1002/ana.410430321
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- Arenas, J., Campos, Y., Ribacoba, R., Martín, M. A., Rubio, J. C., Ablanedo, P., & Cabello, A. (1998). Complex I Defect in muscle from patients with Huntington's disease. Annals of Neurology. https://doi.org/10.1002/ana.410430321
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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.
