The DOI matches its Crossref record; 52 of the 53 references it cites were found in a registry or an index.
Lifespan and mitochondrial control of neurodegeneration
Abstract
We examine the allometric (comparative scaling) relationships between rates of neurodegeneration resulting from equivalent mutations in a diverse group of genes from five mammalian species with different maximum lifespan potentials. In both retina and brain, rates of neurodegeneration vary by as much as two orders of magnitude and are strongly correlated with maximum lifespan potential and rates of formation of mitochondrial reactive oxygen and nitrogen species (RONS). Cell death in these disorders is directly or indirectly regulated by the intrinsic mitochondrial cell death pathway. Mitochondria are the main source of RONS production and integrate cellular stress signals to coordinate the intrinsic pathway. We propose that these two functions are intimately related and that steady-state RONS-mediated signaling or damage to the mitochondrial stress-integration machinery is the principal factor setting the probability of cell death in response to a diverse range of cellular stressors. This provides a new and unifying framework for investigating neurodegenerative disorders.
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
“Aging-associated expression changes in genes associated with mitochondrial function, protein metabolism, and immune response have been identified in several tissues (173) and are consistent with some proposed mechanisms for late-onset neurodegenerative diseases (98, 113, 124, 166).”
From Unraveling a Multifactorial Late-Onset Disease: From Genetic Susceptibility to Disease Mechanisms for Age-Related Macular Degeneration (2009) · cites this paper as [166]
In the citing paper
“Numerous studies have demonstrated a central role of mitochondria and energy homeostasis in the pathogenesis of aging-associated neurodegenerative diseases (98, 157, 166).”
From Unraveling a Multifactorial Late-Onset Disease: From Genetic Susceptibility to Disease Mechanisms for Age-Related Macular Degeneration (2009) · cites this paper as [166]
Citations
Cites
Free radicals in biology and medicine
Gutteridge, J. M. C. (1991) · General Pharmacology The Vascular System · doi:10.1016/0306-3623(91)90478-o · Cited by 17,178
Free Radicals in Biology and Medicine
Halliwell, B., & Gutteridge, J. M. C. (2015) · doi:10.1093/acprof:oso/9780198717478.001.0001 · Cited by 8,990
The Bcl-2 Protein Family: Arbiters of Cell Survival
Adams, J. M., & Cory, S. (1998) · Science · doi:10.1126/science.281.5381.1322 · Cited by 5,334
Danial, N. N., & Korsmeyer, S. J. (2004) · Cell · doi:10.1016/s0092-8674(04)00046-7 · Cited by 4,651
The Free Radical Theory of Aging Matures
BECKMAN, K. B., & AMES, B. N. (1998) · Physiological Reviews · doi:10.1152/physrev.1998.78.2.547 · Cited by 3,879
Scaling: Why is Animal Size so Important?
Schmidt-Nielsen, K. (1984) · doi:10.1017/cbo9781139167826 · Cited by 2,677
Scaling Why is animal size so important?
Schmidt‐Nielsen, K. (1984) · Cited by 2,654
Oxidative stress in neurodegeneration: Cause or consequence?
Andersen, J. K. (2004) · Nature Medicine · doi:10.1038/nrn1434 · Cited by 1,869
Apoptosis in neurodegenerative disorders
Mattson, M. P. (2000) · Nature Reviews Molecular Cell Biology · doi:10.1038/35040009 · Cited by 1,605
Topology of superoxide production from different sites in the mitochondrial electron transport chain
St-Pierre, J., Buckingham, J. A., Roebuck, S. J., & Brand, M. D. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m207217200 · Cited by 1,545
Newmeyer, D. D., & Ferguson-Miller, S. M. (2003) · Cell · doi:10.1016/s0092-8674(03)00116-8 · Cited by 1,272
Redox Regulation of Cellular Signalling
Kamata, H., & Hirata, H. (1999) · Cellular Signalling · doi:10.1016/s0898-6568(98)00037-0 · Cited by 1,162
Cytochrome c release from mitochondria proceeds by a two-step process
Ott, M., Robertson, J. D., Gogvadze, V. G., Zhivotovsky, B., & Orrenius, S. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.241655498 · Cited by 955
In Search of Methuselah: Estimating the Upper Limits to Human Longevity
Olshansky, S. J., Carnes, B. A., & Cassel, C. (1990) · Science · doi:10.1126/science.2237414 · Cited by 645
Boehning, D. F., Patterson, R. L., Sedaghat, L., Glebova, N. O., Kurosaki, T., & Snyder, S. H. (2003) · Nature Cell Biology · doi:10.1038/ncb1063 · Cited by 636
Daitoku, H., Hatta, M., Matsuzaki, H., Aratani, S., Ohshima, T., Miyagishi, M., Nakajima, T., & Fukamizu, A. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0400593101 · Cited by 616
Comfort, A. (1956) · Rinehart eBooks · doi:10.5962/bhl.title.6435 · Cited by 598
A signalling role for 4‐hydroxy‐2‐nonenal in regulation of mitochondrial uncoupling
Echtay, K. S., Esteves, T. C., Pakay, J. L., Jekabsons, M. B., Lambert, A. J., Portero‐Otín, M., Pamplona, R., Vidal‐Puig, A. J., Wang, S., Roebuck, S. J., et al. (2003) · The EMBO Journal · doi:10.1093/emboj/cdg412 · Cited by 597
Prospects for new restorative and neuroprotective treatments in Parkinson's disease
Dunnett, S. B., & Björklund, A. (1999) · Nature · doi:10.1038/399a032 · Cited by 543
Protection of mouse photoreceptors by survival factors in retinal degenerations.
LaVail, M. M., Yasumura, D., Matthes, M. T., Lau-Villacorta, C., Unoki, K., Sung, C. –., & Steinberg, R. H. (1998) · PubMed · Cited by 494
Ku, H., Brunk, U. T., & Sohal, R. S. (1993) · Free Radical Biology and Medicine · doi:10.1016/0891-5849(93)90165-q · Cited by 488
Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease
Turmaine, M., Raza, A., Mahal, A., Mangiarini, L., Bates, G. P., & Davies, S. W. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.110078997 · Cited by 430
Mechanisms Underlying the Cost of Living in Animals
Hulbert, A. J., & Else, P. L. (2000) · Annual Review of Physiology · doi:10.1146/annurev.physiol.62.1.207 · Cited by 403
Superoxide Activates Mitochondrial Uncoupling Protein 2 from the Matrix Side
Echtay, K. S., Murphy, M. P., Smith, R. A. J., Talbot, D. A., & Brand, M. D. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m208262200 · Cited by 388
Jauslin, M. L., Meier, T., Smith, R. A. J., & Murphy, P. M. (2003) · The FASEB Journal · doi:10.1096/fj.03-0240fje · Cited by 358
Pérez‐Campo, R., Lopez‐Torres, M., Cadenas, S., Rojas, C., & Barja, G. (1998) · Journal of Comparative Physiology B · doi:10.1007/s003600050131 · Cited by 356
Apoptosis in Neural Development and Disease
Nijhawan, D., Honarpour, N., & Wang, X. (2000) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.23.1.73 · Cited by 326
Membranes as Possible Pacemakers of Metabolism
Hulbert, A. J., & Else, P. L. (1999) · Journal of Theoretical Biology · doi:10.1006/jtbi.1999.0955 · Cited by 312
Death without caspases, caspases without death
Abraham, M. C., & Shaham, S. (2004) · Trends in Cell Biology · doi:10.1016/j.tcb.2004.03.002 · Cited by 307
A one-hit model of cell death in inherited neuronal degenerations
Clarke, G. D., Collins, R. A., Leavitt, B. R., Andrews, D. F., Hayden, M. R., Lumsden, C. J., & McInnes, R. R. (2000) · Nature · doi:10.1038/35018098 · Cited by 303
Self-citationDisease sequence from mutant rhodopsin allele to rod and cone photoreceptor degeneration in man
Cideciyan, A. V., Hood, D. C., Huang, Y., Banin, E., Li, Z., Stone, E. M., Milam, A. H., & Jacobson, S. G. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.12.7103 · Cited by 278
Self-citationMembrane Fatty Acid Unsaturation, Protection against Oxidative Stress, and Maximum Life Span
Pamplona, R., Barja, G., & Portero‐Otín, M. (2002) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.2002.tb02118.x · Cited by 264
Mitochondrial Free Radical Production and Aging in Mammals and Birdsa
Barja, G. (1998) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1998.tb09905.x · Cited by 246
Apoptotic cell death in retinal degenerations
Remé, C. E., Grimm, C., Hafezi, F., Marti, A., & Wenzel, A. (1998) · Progress in Retinal and Eye Research · doi:10.1016/s1350-9462(98)00009-3 · Cited by 246
Ku, H., & Sohal, R. S. (1993) · Mechanisms of Ageing and Development · doi:10.1016/0047-6374(93)90132-b · Cited by 240
Molecular Genetics of Human Retinal Disease
Rattner, A., Sun, H., & Nathans, J. (1999) · Annual Review of Genetics · doi:10.1146/annurev.genet.33.1.89 · Cited by 228
Porter, R. K., Hulbert, A. J., & Brand, M. D. (1996) · American Journal of Physiology-Regulatory, Integrative and Comparative Physiology · doi:10.1152/ajpregu.1996.271.6.r1550 · Cited by 211
Hydrogen peroxide production by liver mitochondria in different species
Sohal, R. S., Svensson, I., & Brunk, U. T. (1990) · Mechanisms of Ageing and Development · doi:10.1016/0047-6374(90)90039-i · Cited by 172
Superoxide anion radical production in different animal species
Sohal, R. S., Svensson, I., Sohal, B. H., & Brunk, U. T. (1989) · Mechanisms of Ageing and Development · doi:10.1016/0047-6374(89)90096-1 · Cited by 162
K., R. P., Altman, P. L., & Dittmer, D. S. (1975) · Mycologia · doi:10.2307/3758352 · Cited by 156
Turner, N. A., Else, P. L., & Hulbert, A. J. (2003) · Die Naturwissenschaften · doi:10.1007/s00114-003-0470-z · Cited by 151
Pacione, L. R., Szego, M. J., Ikeda, S., Nishina, P. M., & McInnes, R. R. (2003) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.26.041002.131416 · Cited by 145
Self-citationAutophagy and Caspases: A New Cell Death Program
Li, Y., Lenardo, M. J., & Baehrecke, E. H. (2004) · Cell Cycle · doi:10.4161/cc.3.9.1097 · Cited by 124
Rhodopsin mutations in inherited retinal dystrophies and dysfunctions
Gal, A., Apfelstedt-Sylla, E., Janecke, A., & Zrennert, E. (1997) · Progress in Retinal and Eye Research · doi:10.1016/s1350-9462(96)00021-3 · Cited by 119
National Research Council (1997) · National Academies Press eBooks · doi:10.17226/5740 · Cited by 109
Inherited neurodegenerative diseases: the one-hit model of neurodegeneration
Clarke, G. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.20.2269 · Cited by 69
Molecular ophthalmology: an update on animal models for retinal degenerations and dystrophies
Hafezi, F. (2000) · British Journal of Ophthalmology · doi:10.1136/bjo.84.8.922 · Cited by 60
Identifying Retinal Disease Genes: How Far Have We Come, How Far Do We Have to Go?
Daiger, S. P. (2003) · Novartis Foundation symposium · doi:10.1002/0470092645.ch3 · Cited by 55
Oxidative stress and aging – the use of superoxide dismutase/catalase mimetics to extend lifespan
Sampayo, J. N., Gill, M. S., & Lithgow, G. J. (2003) · Biochemical Society Transactions · doi:10.1042/bst0311305 · Cited by 53
Apoptosis: Letting Slip The Dogs Of War
Wolf, B. B., & Green, D. R. (2002) · Current Biology · doi:10.1016/s0960-9822(02)00736-4 · Cited by 41
Tipping the Balance toward Longevity
Antebi, A. (2004) · Developmental Cell · doi:10.1016/s1534-5807(04)00068-1 · Cited by 12
A one-hit model of cell death in inherited neuronal degenerations
Bortolotto, Z. A., Clarke, V. R. J., Delany, C. M., Parry, M., Smolders, I. J., Vignes, M., Ho, K. H., Miu, P., Brinton, B. T., Fantaske, R. P., et al. (2001) · Cited by 9
Flagged first, then by citations
1 of the 53 references couldn’t be matched to a known paper.
Research works printed but not linked: 1
- Shiva, S. et al. Redox signalling: from nitric oxide to oxidised lipids. in Free Radicals: Enzymology, Signalling and Disease (eds. Cooper, C.E., Wilson, M.T. & Darley-Usmar, V.M.) 107–120 (Portland, London, 2004).
Cited by
Sorted by citations
Targeting Antioxidants to Mitochondria by Conjugation to Lipophilic Cations
Murphy, M. P., & Smith, R. A. J. (2007) · Annual Review of Pharmacology and Toxicology · doi:10.1146/annurev.pharmtox.47.120505.105110 · Cited by 1,173
Photoreceptor degeneration: genetic and mechanistic dissection of a complex trait
Wright, A. F., Chakarova, C. F., El-Aziz, M. M. A., & Bhattacharya, S. S. (2010) · Nature Reviews Genetics · doi:10.1038/nrg2717 · Cited by 668
Self-citationTargeting an antioxidant to mitochondria decreases cardiac ischemia-reperfusion injury
Adlam, V. J., Harrison, J. C., Porteous, C. M., James, A. M., Smith, R. A. J., Murphy, M. P., & Sammut, I. A. (2005) · The FASEB Journal · doi:10.1096/fj.05-3718com · Cited by 618
The PerR transcription factor senses H2O2 by metal-catalysed histidine oxidation
Lee, J.-W., & Helmann, J. D. (2006) · Nature · doi:10.1038/nature04537 · Cited by 547
Mitochondrial Disorders in the Nervous System
DiMauro, S., & Schon, E. A. (2008) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.30.051606.094302 · Cited by 541
Metadata
- Authors
- Wright, A. F., Jacobson, S. G., Cideciyan, A. V., Roman, A. J., Shu, X., Vlachantoni, D., McInnes, R. R., & Riemersma, R. A.
- Affiliations
- Western General Hospital (GB)
- University of Pennsylvania (US)
- University of Toronto (CA)
- UiT The Arctic University of Norway (NO)
- Corresponding author
- Wright, Alan F
- Venue
- Nature Genetics
- Published
- 2004-10-28
- Volume
- 36
- Issue
- 11
- Pages
- 1153-1158
- ISSN
- 1061-4036 (ISSN-L)
- 1546-1718
- OpenAlex
- W2050863429
- Cited by
- 111
- References
- 53
- Citations by year
- 2026: 4 · 2025: 3 · 2024: 2 · 2023: 5 · 2022: 4 · 2021: 6 · 2020: 8 · 2019: 3 · 2018: 7 · 2017: 5 · 2016: 3 · 2015: 5 · 2014: 5 · 2013: 3 · 2012: 3
- FWCI
- 0.46
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Genetics (publishedVersion)
- PubMed (publishedVersion)
- ResearchOnline (Glasgow Caledonian University) (acceptedVersion)
- Funders
- Macula Vision Research Foundation
- National Institutes of Health
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Aging › Genetics, Aging, and Longevity in Model Organisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Cell death mechanisms and regulation
- Keywords
- neurodegeneration, lifespan, allometric scaling, retina, brain, mammalian species comparison, genetic mutations
- MeSH terms
- Animals, Brain, Humans, Life Expectancy, Mitochondria, Nerve Degeneration, Retina, Species Specificity, Cell Death, Reactive Oxygen Species, Oxidative Stress, Neurodegenerative Diseases, Reactive Nitrogen Species
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