The Biology of Proteostasis in Aging and Disease

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

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Protein Misfolding, Functional Amyloid, and Human Disease

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The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

Walter, P., & Ron, D. (2011) · Science · doi:10.1126/science.1209038 · Cited by 5,897

Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

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Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice

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Loss of autophagy in the central nervous system causes neurodegeneration in mice

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STRUCTURE AND FUNCTIONS OF THE 20S AND 26S PROTEASOMES

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The amyloid state and its association with protein misfolding diseases.

Knowles, T. P. J., Vendruscolo, M., & Dobson, C. M. (2014) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3810 · Cited by 2,382

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

Ravikumar, B., Vacher, C., Berger, Z., Davies, J. E., Luo, S., Oroz, L. G., Scaravilli, F., Easton, D. F., Duden, R., O'Kane, C. J., et al. (2004) · Nature Genetics · doi:10.1038/ng1362 · Cited by 2,347

Impaired Degradation of Mutant α-Synuclein by Chaperone-Mediated Autophagy

Cuervo, A. M., Stefanis, L., Fredenburg, R., Lansbury, P. T., & Sulzer, D. (2004) · Science · doi:10.1126/science.1101738 · Cited by 2,024

Mammalian autophagy: core molecular machinery and signaling regulation

Yang, Z., & Klionsky, D. J. (2010) · Current Opinion in Cell Biology · doi:10.1016/j.ceb.2009.11.014 · Cited by 1,956

Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagation

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HSP90 at the hub of protein homeostasis: emerging mechanistic insights

Taipale, M., Jarosz, D. F., & Lindquist, S. (2010) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm2918 · Cited by 1,818

Recognition and Processing of Ubiquitin-Protein Conjugates by the Proteasome

Finley, D. (2009) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.78.081507.101607 · Cited by 1,764

The HSP70 chaperone machinery: J proteins as drivers of functional specificity

Kampinga, H. H., & Craig, E. A. (2010) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm2941 · Cited by 1,760

Molecular Chaperone Functions in Protein Folding and Proteostasis

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A multiubiquitin chain is confined to specific lysine in a targeted short-lived protein

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Heat shock factors: integrators of cell stress, development and lifespan

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Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

Watts, G. D. J., Wymer, J., Kovach, M. J., Mehta, S. G., Mumm, S., Darvish, D., Pestronk, A., Whyte, M. P., & Kimonis, V. E. (2004) · Nature Genetics · doi:10.1038/ng1332 · Cited by 1,391

UBE3A/E6-AP mutations cause Angelman syndrome

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Excess brain protein oxidation and enzyme dysfunction in normal aging and in Alzheimer disease.

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Repression of Heat Shock Transcription Factor HSF1 Activation by HSP90 (HSP90 Complex) that Forms a Stress-Sensitive Complex with HSF1

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Chaperone Suppression of α-Synuclein Toxicity in a Drosophila Model for Parkinson's Disease

Auluck, P. K., Chan, H. Y. E., Trojanowski, J. Q., Lee, V. M.-Y., & Bonini, N. M. (2002) · Science · doi:10.1126/science.1067389 · Cited by 1,227

Lysosomal Proteolysis and Autophagy Require Presenilin 1 and Are Disrupted by Alzheimer-Related PS1 Mutations

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Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

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Biological and Chemical Approaches to Diseases of Proteostasis Deficiency

Powers, E. T., Morimoto, R. I., Dillin, A., Kelly, J. W., & Balch, W. E. (2009) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.052308.114844 · Cited by 1,149

Autophagy Induction and Autophagosome Clearance in Neurons: Relationship to Autophagic Pathology in Alzheimer's Disease

Boland, B., Kumar, A., Lee, S., Platt, F. M., Wegiel, J., Yu, W. H., & Nixon, R. A. (2008) · Journal of Neuroscience · doi:10.1523/jneurosci.0800-08.2008 · Cited by 1,119

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

Ravikumar, B. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.9.1107 · Cited by 1,099

The Cell Non-Autonomous Nature of Electron Transport Chain-Mediated Longevity

Durieux, J., Wolff, S., & Dillin, A. (2011) · Cell · doi:10.1016/j.cell.2010.12.016 · Cited by 1,059

The co-chaperone CHIP regulates protein triage decisions mediated by heat-shock proteins

Connell, P. M., Ballinger, C. A., Jiang, J., Wu, Y., Thompson, L. J., Höhfeld, J., & Patterson, C. (2000) · Nature Cell Biology · doi:10.1038/35050618 · Cited by 1,002

Role of Rpn11 Metalloprotease in Deubiquitination and Degradation by the 26 S Proteasome

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The changing scene of amyotrophic lateral sclerosis

Robberecht, W., & Philips, T. (2013) · Nature Reviews Neuroscience · doi:10.1038/nrn3430 · Cited by 957

Chaperone-mediated autophagy: a unique way to enter the lysosome world

Kaushik, S., & Cuervo, A. M. (2012) · Trends in Cell Biology · doi:10.1016/j.tcb.2012.05.006 · Cited by 905

Enhancement of proteasome activity by a small-molecule inhibitor of USP14

Lee, B.-H., Lee, M. J., Park, S., Oh, D.-C., Elsasser, S., Chen, P.-C., Gartner, C., Dimova, N., Hanna, J., Gygi, S. P., et al. (2010) · Nature · doi:10.1038/nature09299 · Cited by 893

Cargo recognition failure is responsible for inefficient autophagy in Huntington’s disease

Martinez-Vicente, M., Talloczy, Z., Wong, E., Tang, G., Koga, H., Kaushik, S., de Vries, R., Arias, E., Harris, S., Sulzer, D., et al. (2010) · Nature Neuroscience · doi:10.1038/nn.2528 · Cited by 885

Some like it hot: the structure and function of small heat-shock proteins

Haslbeck, M., Franzmann, T. M., Weinfurtner, D., & Büchner, J. (2005) · Nature Structural & Molecular Biology · doi:10.1038/nsmb993 · Cited by 858

Impaired Proteasome Function in Alzheimer's Disease

Keller, J. N., Hanni, K. B., & Markesbery, W. R. (2000) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2000.0750436.x · Cited by 848

Opposing activities protect against age-onset proteotoxicity

Cohen, E., Bieschke, J., Perciavalle, R. M., Kelly, J. W., & Dillin, A. (2006) · Science · doi:10.1126/science.1124646 · Cited by 843

Heat Shock Factor 1 Is a Powerful Multifaceted Modifier of Carcinogenesis

Dai, C., Whitesell, L. J., Rogers, A. B., & Lindquist, S. L. (2007) · Cell · doi:10.1016/j.cell.2007.07.020 · Cited by 843

α-Synuclein propagates from mouse brain to grafted dopaminergic neurons and seeds aggregation in cultured human cells

Hansen, C., Angot, E., Bergström, A.-L., Steiner, J. A., Pieri, L., Paul, G., Outeiro, T. F., Melki, R., Kallunki, P., Fog, K., et al. (2011) · Journal of Clinical Investigation · doi:10.1172/jci43366 · Cited by 842

Yeast heat shock factor is an essential DNA-binding protein that exhibits temperature-dependent phosphorylation

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α-Synuclein impairs macroautophagy: implications for Parkinson’s disease

Winslow, A. R., Chen, C.-W., Corrochano, S., Acevedo-Arozena, A., Gordon, D. E., Peden, A. A., Lichtenberg, M., Menzies, F. M., Ravikumar, B., Imarisio, S., et al. (2010) · Journal of Cell Biology · doi:10.1083/jcb.201003122 · Cited by 825

The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans

Morley, J. F., Brignull, H. R., Weyers, J. J., & Morimoto, R. I. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.152161099 · Cited by 811

Novel targets for Huntington's disease in an mTOR-independent autophagy pathway

Williams, A., Sarkar, S., Cuddon, P., Ttofi, E. K., Saiki, S., Siddiqi, F. H., Jahreiss, L., Fleming, A., Pask, D., Goldsmith, P., et al. (2008) · Nature Chemical Biology · doi:10.1038/nchembio.79 · Cited by 800

Stress-activated cap’n’collar transcription factors in aging and human disease

Sykiotis, G. P., & Bohmann, D. (2010) · Science Signaling · doi:10.1126/scisignal.3112re3 · Cited by 776

A cryptic protease couples deubiquitination and degradation by the proteasome

Yao, T., & Cohen, R. E. (2002) · Nature · doi:10.1038/nature01071 · Cited by 727

Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

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Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

Hetz, C., & Mollereau, B. (2014) · Nature Reviews Neuroscience · doi:10.1038/nrn3689 · Cited by 684

Proteostasis impairment in protein-misfolding and -aggregation diseases

Hipp, M. S., Park, S.-H., & Hartl, F. U. (2014) · Trends in Cell Biology · doi:10.1016/j.tcb.2014.05.003 · Cited by 683

Molecular chaperones as HSF1-specific transcriptional repressors.Preprint

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Dopamine-modified α-synuclein blocks chaperone-mediated autophagy

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Rapamycin alleviates toxicity of different aggregate-prone proteins

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FOXO/4E-BP Signaling in Drosophila Muscles Regulates Organism-wide Proteostasis during Aging

Demontis, F., & Perrimon, N. (2010) · Cell · doi:10.1016/j.cell.2010.10.007 · Cited by 656

Proteasomal function is impaired in substantia nigra in Parkinson’s disease

McNaught, K. S. P., & Jenner, P. (2001) · Neuroscience Letters · doi:10.1016/s0304-3940(00)01701-8 · Cited by 653

A Ribosome-Bound Quality Control Complex Triggers Degradation of Nascent Peptides and Signals Translation Stress

Brandman, O., Stewart-Ornstein, J., Wong, D., Larson, A. G., Williams, C. C., Li, G., Zhou, S., King, D., Shen, P. S., Weibezahn, J., et al. (2012) · Cell · doi:10.1016/j.cell.2012.10.044 · Cited by 650

Collapse of proteostasis represents an early molecular event in Caenorhabditis elegans aging

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Progressive disruption of cellular protein folding in models of polyglutamine diseases

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Stress-inducible regulation of heat shock factor 1 by the deacetylase SIRT1

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Widespread protein aggregation as an inherent part of aging in C. elegans

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Small molecules enhance autophagy and reduce toxicity in Huntington's disease models

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XBP-1 Is a Cell-Nonautonomous Regulator of Stress Resistance and Longevity

Taylor, R. C., & Dillin, A. G. (2013) · Cell · doi:10.1016/j.cell.2013.05.042 · Cited by 585

A chaperome subnetwork safeguards proteostasis in aging and neurodegenerative disease

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Mutant small heat-shock protein 27 causes axonal Charcot-Marie-Tooth disease and distal hereditary motor neuropathy

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The Sizes of Peptides Generated from Protein by Mammalian 26 and 20 S Proteasomes

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HSF1 is required for extra‐embryonic development, postnatal growth and protection during inflammatory responses in mice

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Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice

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Global changes to the ubiquitin system in Huntington's disease

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Advances in the genetics of Parkinson disease

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Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells

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Editing of ubiquitin conjugates by an isopeptidase in the 26S proteasome

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Activation of Human Heat Shock Genes Is Accompanied by Oligomerization, Modification, and Rapid Translocation of Heat Shock Transcription Factor HSF1

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Deciphering the mechanism underlying late-onset Alzheimer disease

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Autosomal dominant congenital cataract associated with a missense mutation in the human alpha crystallin gene CRYAA

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Timing Requirements for Insulin/IGF-1 Signaling in C. elegans

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Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease

Sittler, A. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.12.1307 · Cited by 442

Human αB-Crystallin Mutation Causes Oxido-Reductive Stress and Protein Aggregation Cardiomyopathy in Mice

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ARSACS, a spastic ataxia common in northeastern Québec, is caused by mutations in a new gene encoding an 11.5-kb ORF

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Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

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Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates

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Analysis of the Role of Heat Shock Protein (Hsp) Molecular Chaperones in Polyglutamine Disease

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RPN-6 determines C. elegans longevity under proteotoxic stress conditions

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A Quantitative Chaperone Interaction Network Reveals the Architecture of Cellular Protein Homeostasis Pathways

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17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration

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Increased proteasome activity in human embryonic stem cells is regulated by PSMD11

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Huntington's disease: underlying molecular mechanisms and emerging concepts

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Cooperation of a ubiquitin domain protein and an E3 ubiquitin ligase during chaperone/proteasome coupling

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Hereditary Spastic Paraplegia SPG13 Is Associated with a Mutation in the Gene Encoding the Mitochondrial Chaperonin Hsp60

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PolyQ Proteins Interfere with Nuclear Degradation of Cytosolic Proteins by Sequestering the Sis1p Chaperone

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Regulation of the Cellular Heat Shock Response in Caenorhabditis elegans by Thermosensory Neurons

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Deubiquitinating Enzyme Ubp6 Functions Noncatalytically to Delay Proteasomal Degradation

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Widespread Regulation of Translation by Elongation Pausing in Heat Shock

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The gene for Machado–Joseph disease maps to human chromosome 14q

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Autophagy and Lipid Metabolism Coordinately Modulate Life Span in Germline-less C. elegans

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