The DOI matches its Crossref record; 47 of the 47 references it cites were found in a registry or an index.
Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
Abstract
Here we report that chromogranins, components of neurosecretory vesicles, interact with mutant forms of superoxide dismutase (SOD1) that are linked to amyotrophic lateral sclerosis (ALS), but not with wild-type SOD1. This interaction was confirmed by yeast two-hybrid screen and by co-immunoprecipitation assays using either lysates from Neuro2a cells coexpressing chromogranins and SOD1 mutants or lysates from spinal cord of ALS mice. Confocal and immunoelectron microscopy revealed a partial colocalization of mutant SOD1 with chromogranins in spinal cord of ALS mice. Mutant SOD1 was also found in immuno-isolated trans-Golgi network and in microsome preparations, suggesting that it can be secreted. Indeed we report evidence that chromogranins may act as chaperone-like proteins to promote secretion of SOD1 mutants. From these results, and our finding that extracellular mutant SOD1 can trigger microgliosis and neuronal death, we propose a new ALS pathogenic model based on the toxicity of secreted SOD1 mutants.
Overview
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
In the citing paper
“Protein aggregates released into the extracellular space together with DAMPs released from damaged neurons induce microglia activation and proinflammatory cytokines releasing.20,83,84”
From Role of neuroinflammation in neurodegeneration development (2023) · cites this paper as [84]
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
Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation
Gurney, M. E., Pu, H., Chiu, A. Y., Dal Canto, M. C., Polchow, C. Y., Alexander, D. D., Caliendo, J., Hentati, A., Kwon, Y. W., Deng, H.-X., et al. (1994) · Science · doi:10.1126/science.8209258 · Cited by 4,143
From charcot to lou gehrig: deciphering selective motor neuron death in als
Cleveland, D. W., & Rothstein, J. D. (2001) · Nature reviews. Neuroscience · doi:10.1038/35097565 · Cited by 1,389
Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice
Clement, A. M., Nguyen, M. D., Roberts, E. A., Garcia, M. L., Boillée, S., Rule, M., McMahon, A. P., Doucette, W. T., Siwek, D. F., Ferrante, R. J., et al. (2003) · Science · doi:10.1126/science.1086071 · Cited by 1,131
Self-citationZhu, S., Stavrovskaya, I. G., Drozda, M., Kim, B. Y. S., Ona, V. O., Li, M., Sarang, S. S., Liu, A. S., Hartley, D. M., Wu, D. C., et al. (2002) · Nature · doi:10.1038/417074a · Cited by 1,069
The Chromogranin–Secretogranin Family
Taupenot, L., Harper, K. L., & O’Connor, D. T. (2003) · New England Journal of Medicine · doi:10.1056/nejmra021405 · Cited by 819
Altered Reactivity of Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis
Wiedau‐Pazos, M., Goto, J. J., Rabizadeh, S., Gralla, E. B., Roe, J. A., Lee, M. K., Valentine, J. S., & Bredesen, D. E. (1996) · Science · doi:10.1126/science.271.5248.515 · Cited by 720
Johnston, J. A., Dalton, M. J., Gurney, M. E., & Kopito, R. R. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.220417997 · Cited by 616
The mystery of nonclassical protein secretion
Nickel, W. (2003) · European Journal of Biochemistry · doi:10.1046/j.1432-1033.2003.03577.x · Cited by 586
Pramatarova, A., Laganière, J., Roussel, J., Brisebois, K., & Rouleau, G. A. (2001) · The Journal of Neuroscience · doi:10.1523/jneurosci.21-10-03369.2001 · Cited by 482
Flagged first, then by citations
Cited by
Sorted by citations
Mechanisms underlying inflammation in neurodegeneration
Glass, C. K., Saijo, K., Winner, B., Marchetto, M. C., & Gage, F. H. (2010) · Cell · doi:10.1016/j.cell.2010.02.016 · Cited by 3,580
Decoding ALS: from genes to mechanism
Taylor, J. P., Brown, R. H., & Cleveland, D. W. (2016) · Nature · doi:10.1038/nature20413 · Cited by 2,120
Transmission and spreading of tauopathy in transgenic mouse brain
Clavaguera, F., Bolmont, T., Crowther, R. A., Abramowski, D., Frank, S., Probst, A., Fraser, G., Stalder, A. K., Beibel, M., Staufenbiel, M., et al. (2009) · Nature Cell Biology · doi:10.1038/ncb1901 · Cited by 1,803
Role of neuroinflammation in neurodegeneration development
Zhang, W., Xiao, D., Mao, Q., & Xia, H. (2023) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-023-01486-5 · Cited by 1,668
“Protein aggregates released into the extracellular space together with DAMPs released from damaged neurons induce microglia activation and proinflammatory cytokines releasing.(20,83,84) Persistent inflammation damages neurons directly and exacerbates ALS(78,85) (Fig.”
BackgroundALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors
Boillée, S., Velde, C. V., & Cleveland, D. W. (2006) · Neuron · doi:10.1016/j.neuron.2006.09.018 · Cited by 1,476
Metadata
- Authors
- Urushitani, M., Sik, A., Sakurai, T., Nukina, N., Takahashi, R., & Julien, J.-P.
- Affiliations
- Centre hospitalier de l'Université Laval (CA)
- Université Laval (CA)
- RIKEN Center for Brain Science (JP)
- Corresponding author
- Julien, Jean-Pierre
- Venue
- Nature Neuroscience
- Published
- 2005-12-20
- Volume
- 9
- Issue
- 1
- Pages
- 108-118
- ISSN
- 1097-6256 (ISSN-L)
- 1546-1726
- OpenAlex
- W2034651824
- Semantic Scholar
- 6da103b1c172cd68887144303844e02b0c5552ff
- Cited by
- 419
- References
- 47
- Citations by year
- 2026: 4 · 2025: 8 · 2024: 12 · 2023: 10 · 2022: 8 · 2021: 9 · 2020: 14 · 2019: 10 · 2018: 13 · 2017: 16 · 2016: 20 · 2015: 25 · 2014: 23 · 2013: 35 · 2012: 28
- FWCI
- 15.58
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- University of Birmingham Research Portal (University of Birmingham) (submittedVersion)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- chromogranin, superoxide dismutase 1, SOD1 mutations, amyotrophic lateral sclerosis, protein secretion, neurosecretory granules, chaperone-like protein, trans-Golgi network, microglial activation, neuronal cell death, ALS pathogenesis, spinal cord
- MeSH terms
- Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, Animals, Astrocytes, Cells, Cultured, Chlorocebus aethiops, Chromogranins, Fluorescent Antibody Technique, Gliosis, Humans, Interneurons, Mice, Transgenic, Microsomes, Motor Neurons, Mutation, Plasmids, Spinal Cord, Subcellular Fractions, Superoxide Dismutase, Blotting, Western, Microscopy, Immunoelectron, 3T3 Cells, Protein Folding, Molecular Chaperones, COS Cells, Immunoprecipitation, Mice, Chromogranin A, Proteasome Inhibitors
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
