| P50 | author | David Vilchez | Q46938838 |
| P2093 | author name string | Ricardo Gutierrez-Garcia | |
| | Seda Koyuncu | |
| | Azra Fatima | |
| P2860 | cites work | The ubiquitin-proteasome proteolytic pathway: destruction for the sake of construction | Q24292709 |
| | Increased proteasome activity in human embryonic stem cells is regulated by PSMD11 | Q24298544 |
| | Aging as an event of proteostasis collapse | Q24630961 |
| | The many faces of autophagy dysfunction in Huntington's disease: from mechanism to therapy | Q26823365 |
| | Chaperone machines for protein folding, unfolding and disaggregation | Q27026110 |
| | The molecular architecture of the eukaryotic chaperonin TRiC/CCT | Q27678510 |
| | Mechanisms underlying ubiquitination | Q27860656 |
| | Mechanism of the eukaryotic chaperonin: protein folding in the chamber of secrets. | Q27919668 |
| | Systems analyses reveal two chaperone networks with distinct functions in eukaryotic cells | Q27932599 |
| | PolyQ proteins interfere with nuclear degradation of cytosolic proteins by sequestering the Sis1p chaperone | Q27937491 |
| | Huntingtin functions as a scaffold for selective macroautophagy | Q28118022 |
| | The hallmarks of aging | Q28131641 |
| | Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity | Q28143597 |
| | Huntingtin aggregation and toxicity in Huntington's disease | Q28204163 |
| | Molecular chaperones in the cytosol: from nascent chain to folded protein | Q28205903 |
| | Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation | Q28246858 |
| | A chaperome subnetwork safeguards proteostasis in aging and neurodegenerative disease | Q28252463 |
| | Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease | Q28261987 |
| | The roles of intracellular protein-degradation pathways in neurodegeneration | Q28269322 |
| | Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin | Q28276832 |
| | Normal huntingtin function: an alternative approach to Huntington's disease | Q28281904 |
| | Regulation of proteasome activity in health and disease | Q28392708 |
| | Microautophagy of cytosolic proteins by late endosomes | Q28509224 |
| | The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes | Q28580600 |
| | Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease | Q28588314 |
| | p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death | Q29547297 |
| | Regulation mechanisms and signaling pathways of autophagy | Q29547416 |
| | Recognition and processing of ubiquitin-protein conjugates by the proteasome | Q29547616 |
| | Molecular chaperones in protein folding and proteostasis | Q29547715 |
| | Autophagy genes are essential for dauer development and life-span extension in C. elegans | Q29614180 |
| | Gene expression profile of aging and its retardation by caloric restriction | Q29614554 |
| | Impairment of the ubiquitin-proteasome system by protein aggregation | Q29614556 |
| | Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging | Q29614783 |
| | The role of autophagy in neurodegenerative disease | Q29614834 |
| | Autophagy and aging | Q29616002 |
| | Ubiquitin: structures, functions, mechanisms | Q29616462 |
| | Eaten alive: a history of macroautophagy | Q29617841 |
| | Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain | Q29617982 |
| | Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy | Q29622819 |
| | IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. | Q30435758 |
| | Small molecule inducers of heat-shock response reduce polyQ-mediated huntingtin aggregation. A possible therapeutic strategy. | Q50675323 |
| | Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state. | Q52573641 |
| | Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. | Q52574703 |
| | Identification of genes that modify ataxin-1-induced neurodegeneration. | Q52584075 |
| | Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila. | Q52584268 |
| | The ubiquitin-proteasome pathway in Huntington's disease. | Q55433686 |
| | NBR1 and p62 as cargo receptors for selective autophagy of ubiquitinated targets | Q57269938 |
| | Chaperone-Mediated Autophagy | Q57376122 |
| | Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model | Q79948907 |
| | Monitoring activity and inhibition of 26S proteasomes with fluorogenic peptide substrates | Q81453964 |
| | Huntington's Disease | Q83728053 |
| | Barcoding heat shock proteins to human diseases: looking beyond the heat shock response | Q38203487 |
| | Autophagy in Huntington disease and huntingtin in autophagy | Q38256703 |
| | The role of protein clearance mechanisms in organismal ageing and age-related diseases. | Q38284376 |
| | Neuronal properties, in vivo effects, and pathology of a Huntington's disease patient-derived induced pluripotent stem cells | Q38462208 |
| | Amyloid Fibres: Inert End-Stage Aggregates or Key Players in Disease? | Q38626285 |
| | Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation | Q38720406 |
| | The Biology of Huntingtin. | Q38759081 |
| | Cellular Homeostasis and Aging | Q38799467 |
| | Polyglutamine tracts regulate beclin 1-dependent autophagy | Q38818577 |
| | In vivo aspects of protein folding and quality control | Q38882864 |
| | Embryonic stem cells: a novel paradigm to study proteostasis? | Q38891967 |
| | Label-free quantitative proteomics reveals the dynamics of proteasome complexes composition and stoichiometry in a wide range of human cell lines. | Q38997541 |
| | Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress | Q39064401 |
| | Insulin/IGF-1 signaling regulates proteasome activity through the deubiquitinating enzyme UBH-4. | Q39139202 |
| | Proteostatic and Metabolic Control of Stemness | Q39286039 |
| | A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation | Q39738894 |
| | Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic | Q40065738 |
| | Chaperones: needed for both the good times and the bad times | Q40183775 |
| | BAG-1 associates with the polyglutamine-expanded huntingtin aggregates. | Q40444523 |
| | Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation. | Q40461698 |
| | Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes. | Q40467483 |
| | Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation | Q40540661 |
| | Huntington's disease: update and review of neuropsychiatric aspects | Q40612866 |
| | Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function. | Q40658225 |
| | Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. | Q40704833 |
| | Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release | Q40808988 |
| | Interaction of Hsp70 chaperones with substrates | Q41465900 |
| | Genetic suppression of polyglutamine toxicity in Drosophila | Q41724127 |
| | Disease-specific phenotypes in dopamine neurons from human iPS-based models of genetic and sporadic Parkinson's disease. | Q41964090 |
| | Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein | Q42598739 |
| | Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes | Q42714345 |
| | Genetic evidence linking age-dependent attenuation of the 26S proteasome with the aging process | Q43179672 |
| | Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice | Q43670927 |
| | Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. | Q44286601 |
| | Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila | Q44607268 |
| | Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins | Q44833524 |
| | Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach | Q44872348 |
| | Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease | Q45290871 |
| | Autophagy regulates the processing of amino terminal huntingtin fragments | Q45291371 |
| | Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo | Q45294913 |
| | Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. | Q45296540 |
| | Inhibition of 26S proteasome activity by huntingtin filaments but not inclusion bodies isolated from mouse and human brain | Q45301604 |
| | Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation | Q45304513 |
| | Primary Cilium-Autophagy-Nrf2 (PAN) Axis Activation Commits Human Embryonic Stem Cells to a Neuroectoderm Fate. | Q46139377 |
| | RPN-6 determines C. elegans longevity under proteotoxic stress conditions | Q46942610 |
| | NBR1 cooperates with p62 in selective autophagy of ubiquitinated targets. | Q47929108 |
| | Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. | Q47991885 |
| | Huntington disease | Q48465200 |
| | The S/T-Rich Motif in the DNAJB6 Chaperone Delays Polyglutamine Aggregation and the Onset of Disease in a Mouse Model. | Q48763720 |
| | Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3). | Q48900156 |
| | Protein Homeostasis in Models of Aging and Age-Related Conformational Disease | Q36109387 |
| | Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation | Q36255989 |
| | siRNA screen identifies QPCT as a druggable target for Huntington's disease | Q36416732 |
| | Amyloid-binding compounds maintain protein homeostasis during ageing and extend lifespan | Q36723364 |
| | Autophagy and neurodegeneration: when the cleaning crew goes on strike | Q36760662 |
| | Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins | Q37109961 |
| | Huntington's disease: degradation of mutant huntingtin by autophagy | Q37220135 |
| | VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease | Q37223339 |
| | Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. | Q37323312 |
| | Somatic increase of CCT8 mimics proteostasis of human pluripotent stem cells and extends C. elegans lifespan | Q37465080 |
| | Ubiquilin-1 overexpression increases the lifespan and delays accumulation of Huntingtin aggregates in the R6/2 mouse model of Huntington's disease | Q37521285 |
| | Inefficient degradation of truncated polyglutamine proteins by the proteasome | Q37592772 |
| | Chaperone-mediated autophagy: selectivity pays off. | Q37621744 |
| | Sulforaphane enhances proteasomal and autophagic activities in mice and is a potential therapeutic reagent for Huntington's disease. | Q37719851 |
| | Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease | Q37776087 |
| | Proteasome and oxidative phoshorylation changes may explain why aging is a risk factor for neurodegenerative disorders | Q37784874 |
| | Proteasome activators | Q37826501 |
| | The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis | Q38018281 |
| | Structural biology of the proteasome | Q38081952 |
| | The proteasome: from basic mechanisms to emerging roles | Q38096863 |
| | Proteostasis and aging of stem cells | Q38149484 |
| | Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease | Q30474580 |
| | Identification of a post-translationally myristoylated autophagy-inducing domain released by caspase cleavage of huntingtin. | Q30578636 |
| | Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells | Q30779186 |
| | Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression | Q30987442 |
| | Formation and toxicity of soluble polyglutamine oligomers in living cells | Q30994681 |
| | Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression | Q31139684 |
| | Proteasome activator enhances survival of Huntington's disease neuronal model cells | Q33275949 |
| | Modulation of heat shock transcription factor 1 as a therapeutic target for small molecule intervention in neurodegenerative disease | Q33526350 |
| | The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactions | Q33693921 |
| | Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregation | Q33694463 |
| | Huntington's disease: from molecular pathogenesis to clinical treatment | Q33773061 |
| | Expanding our understanding of polyglutamine diseases through mouse models | Q33792953 |
| | Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease | Q33814719 |
| | Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone | Q33858200 |
| | Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments | Q33878287 |
| | Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease | Q34114458 |
| | Cellular strategies for controlling protein aggregation | Q34143451 |
| | Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease | Q34168124 |
| | The stress of protein misfolding: from single cells to multicellular organisms | Q34181891 |
| | The heat shock response: systems biology of proteotoxic stress in aging and disease | Q34257283 |
| | Yeast genes that enhance the toxicity of a mutant huntingtin fragment or alpha-synuclein | Q34281429 |
| | Integration of Clearance Mechanisms: The Proteasome and Autophagy | Q34313770 |
| | Systemic exposure to proteasome inhibitors causes a progressive model of Parkinson's disease | Q34331409 |
| | The biology of proteostasis in aging and disease | Q34467650 |
| | Progressive disruption of cellular protein folding in models of polyglutamine diseases. | Q34493281 |
| | Small molecules enhance autophagy and reduce toxicity in Huntington's disease models. | Q34626231 |
| | Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease | Q35084195 |
| | Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils | Q35169533 |
| | A first line of stress defense: small heat shock proteins and their function in protein homeostasis | Q35178845 |
| | The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg. | Q35558459 |
| | Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones | Q35674426 |
| | Pharmacological maintenance of protein homeostasis could postpone age-related disease | Q35836401 |
| | Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease | Q35839766 |
| | The aggravating role of the ubiquitin-proteasome system in neurodegeneration | Q36020204 |
| P275 | copyright license | Creative Commons Attribution | Q6905323 |
| P6216 | copyright status | copyrighted | Q50423863 |
| P433 | issue | 7 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | Huntingtin protein | Q24779247 |
| P577 | publication date | 2017-07-19 | |
| P1433 | published in | International Journal of Molecular Sciences | Q3153277 |
| P1476 | title | Proteostasis of Huntingtin in Health and Disease | |
| P478 | volume | 18 | |