The DOI matches its Crossref record; 36 of the 36 references it cites were found in a registry or an index.
Spastin, the protein mutated in autosomal dominant hereditary spastic paraplegia, is involved in microtubule dynamics
Open accessAbstract
Hereditary spastic paraplegia (HSP) is characterized by progressive weakness and spasticity of the lower limbs, caused by the specific degeneration of the corticospinal tracts, the longest axons in humans. Most cases of the autosomal dominant form of the disease are due to mutations in the SPG4 gene, which encodes spastin, an ATPase belonging to the AAA family. The cellular pathways in which spastin operates and its role in causing degeneration of motor axons are currently unknown. By expressing wild-type or ATPase-defective spastin in several cell types, we now show that spastin interacts dynamically with microtubules. Spastin association with the microtubule cytoskeleton is mediated by the N-terminal region of the protein, and is regulated through the ATPase activity of the AAA domain. Expression of all the missense mutations into the AAA domain, which were previously identified in patients, leads to constitutive binding to microtubules in transfected cells and induces the disappearance of the aster and the formation of thick perinuclear bundles, suggesting a role of spastin in microtubule dynamics. Consistently, wild-type spastin promotes microtubule disassembly in transfected cells. These data suggest that spastin may be involved in microtubule dynamics similarly to the highly homologous microtubule-severing protein, katanin. Impairment of fine regulation of the microtubule cytoskeleton in long axons, due to spastin mutations, may underlie pathogenesis of HSP.
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
Neuwald, A. F., Aravind, L., Spouge, J. L., & Koonin, E. V. (1999) · Genome Research · doi:10.1101/gr.9.1.27 · Cited by 1,828
CLASSIFICATION OF THE HEREDITARY ATAXIAS AND PARAPLEGIAS
Harding, A. E. (1983) · The Lancet · doi:10.1016/s0140-6736(83)92879-9 · Cited by 965
Casari, G., De Fusco, M., Ciarmatori, S., Zeviani, M., Mora, M., Fernandez, P., De Michele, G., Filla, A., Cocozza, S., Marconi, R., et al. (1998) · Cell · doi:10.1016/s0092-8674(00)81203-9 · Cited by 830
Isolation of Monoclonal Antibodies Specific for Human c-myc Proto-Oncogene Product
Evan, G. I., Lewis, G. K., Ramsay, G., & Bishop, J. M. (1985) · Molecular and Cellular Biology · doi:10.1128/mcb.5.12.3610-3616.1985 · Cited by 794
Babst, M. (1998) · The EMBO Journal · doi:10.1093/emboj/17.11.2982 · Cited by 769
Hazan, J., Fonknechten, N., Mavel, D., Paternotte, C., Samson, D., Artiguenave, F., Davoine, C.-S., Cruaud, C., Dürr, A., Wincker, P., et al. (1999) · Nature Genetics · doi:10.1038/15472 · Cited by 634
Identification of katanin, an ATPase that severs and disassembles stable microtubules
McNally, F. J., & Vale, R. D. (1993) · Cell · doi:10.1016/0092-8674(93)90377-3 · Cited by 515
The AAA team: related ATPases with diverse functions
Patel, S. (1998) · Trends in Cell Biology · doi:10.1016/s0962-8924(97)01212-9 · Cited by 515
Whiteheart, S. W., Rossnagel, K., Buhrow, S. A., Brunner, M., Jaenicke, R., & Rothman, J. E. (1994) · The Journal of cell biology · doi:10.1083/jcb.126.4.945 · Cited by 393
Saugier-Veber, P., Munnich, A., Bonneau, D., Rozet, J.-M., Le Merrer, M., Gil, R., & Boespflug-Tanguy, O. (1994) · Nature Genetics · doi:10.1038/ng0394-257 · Cited by 359
Cited by
Microtubule assembly, organization and dynamics in axons and dendrites
Conde, C., & Cáceres, A. (2009) · Nature Reviews Neuroscience · doi:10.1038/nrn2631 · Cited by 1,086
Hereditary spastic paraplegia: clinical features and pathogenetic mechanisms
Salinas, S., Proukakis, C., Crosby, A., & Warner, T. T. (2008) · The Lancet Neurology · doi:10.1016/s1474-4422(08)70258-8 · Cited by 474
Park, S. H., Zhu, P.-P., Parker, R. L., & Blackstone, C. (2010) · Journal of Clinical Investigation · doi:10.1172/jci40979 · Cited by 381
Hereditary spastic paraplegia: Clinical-genetic characteristics and evolving molecular mechanisms
Lo Giudice, T., Lombardi, F., Santorelli, F. M., Kawarai, T., & Orlacchio, A. (2014) · Experimental Neurology · doi:10.1016/j.expneurol.2014.06.011 · Cited by 347
Fink, J. K. (2006) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-996-0011-1 · Cited by 321
Metadata
- Authors
- Errico, A.
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 2002-01-01
- Volume
- 11
- Issue
- 2
- Pages
- 153-163
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2018382970
- Cited by
- 342
- References
- 36
- FWCI
- 9.49
- Type
- journal article
- Language
- en
- Open access
- bronze
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: Spastin, the protein mutated in autosomal dominant hereditary spastic paraplegia, is involved in microtubule dynamics. https://app.cochat.ai/papers/10.1093/hmg/11.2.153
- The paper
- Errico, A. (2002). Spastin, the protein mutated in autosomal dominant hereditary spastic paraplegia, is involved in microtubule dynamics. Human Molecular Genetics. https://doi.org/10.1093/hmg/11.2.153
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.
