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Journal Article
Molecular Cell, ISSN 1097-2765, 10/2009, Volume 36, Issue 1, pp. 15 - 27
The multifunctional, stress-inducible molecular chaperone HSP70 has important roles in aiding protein folding and maintaining protein homeostasis. HSP70... 
PROTEINS | CHEMBIO | CELLCYCLE | CANCER-CELLS | HSP70 | CHAPERONE-MEDIATED AUTOPHAGY | NEGATIVE REGULATION | LYSOSOMAL MEMBRANE | BIOCHEMISTRY & MOLECULAR BIOLOGY | HEAT-SHOCK RESPONSE | APAF-1 APOPTOSOME | P53 PROTEINS | P62 | CELL-DEATH | CELL BIOLOGY | HSP70 Heat-Shock Proteins - antagonists & inhibitors | Microtubule-Associated Proteins - metabolism | Sequestosome-1 Protein | Humans | NF-kappa B - metabolism | Cathepsin L - metabolism | Autophagy - drug effects | DNA-Binding Proteins - metabolism | Caspases - metabolism | Lysosomes - metabolism | Protein Binding - drug effects | Lymphoma - pathology | Protein Interaction Domains and Motifs | Cell Death - drug effects | Lysosomes - drug effects | Cell Survival - drug effects | HSP40 Heat-Shock Proteins - metabolism | Tumor Suppressor Protein p53 - metabolism | Ubiquitin-Protein Ligases - metabolism | HSP70 Heat-Shock Proteins - genetics | Mice, Transgenic | Mice, Inbred Strains | Sulfonamides - pharmacology | Apoptotic Protease-Activating Factor 1 - metabolism | HSP70 Heat-Shock Proteins - metabolism | Transcription Factors - metabolism | Animals | Sulfonamides - therapeutic use | Lymphoma - prevention & control | Sulfonamides - metabolism | Cell Line, Tumor | Mice | Adaptor Proteins, Signal Transducing - metabolism | Protein Multimerization - drug effects | Protein Binding - physiology | Proteins | Heat shock proteins | Analysis | Tumors | Index Medicus
Journal Article
Neuron, ISSN 0896-6273, 04/2013, Volume 78, Issue 1, pp. 65 - 80
Mutations in VCP cause multisystem degeneration impacting the nervous system, muscle, and/or bone. Patients may present with ALS, Parkinsonism, frontotemporal... 
PATHOGENESIS | PARKIN | VALOSIN-CONTAINING-PROTEIN | INCLUSION-BODY MYOPATHY | DROSOPHILA MODEL | FRONTOTEMPORAL DEMENTIA | PAGETS-DISEASE | BONE | NEUROSCIENCES | P97 | DEGENERATION | Embryo, Mammalian | Humans | Ganglia, Spinal - cytology | Drosophila Proteins - metabolism | HSP72 Heat-Shock Proteins - genetics | Protein Tyrosine Phosphatases - genetics | Neurons - ultrastructure | Time Factors | Carbonyl Cyanide m-Chlorophenyl Hydrazone - pharmacology | Neuromuscular Junction - genetics | Neurons - metabolism | Protein-Serine-Threonine Kinases - metabolism | Mitochondrial Membrane Transport Proteins - metabolism | Cell Cycle Proteins - metabolism | Enzyme Inhibitors - pharmacology | Ubiquitin-Protein Ligases - metabolism | Adenosine Triphosphatases - metabolism | Mutation - genetics | Leupeptins - pharmacology | GTP Phosphohydrolases - metabolism | Luminescent Proteins - genetics | Adenosine Triphosphatases - genetics | Ubiquitin-Protein Ligases - genetics | RNA, Small Interfering - metabolism | Immunoprecipitation | Neuromuscular Junction - metabolism | Valosin Containing Protein | Mitochondria - ultrastructure | Transfection | Mitochondria - genetics | Proton Ionophores - pharmacology | Cell Cycle Proteins - genetics | Microscopy, Electron, Transmission | Animals, Genetically Modified | Gene Expression Regulation - genetics | Drosophila | RNA, Small Interfering - pharmacology | Cells, Cultured | Protein-Serine-Threonine Kinases - genetics | Nuclear Proteins - metabolism | Mitochondria - metabolism | Mitochondria - drug effects | Transcription Factors - genetics | Transcription Factors - metabolism | Animals | Proteins - metabolism | Drosophila Proteins - genetics | In Vitro Techniques | Ubiquitination - genetics | Luminescent Proteins - metabolism | Ubiquitin | Analysis | Genomics | Quality control | Amyotrophic lateral sclerosis | Genetic aspects | Mitochondrial DNA | Dementia | Neurons | Parkinsons disease | Biosynthesis | Digital cameras | Kinases | DNA repair | Proteins | Mitochondria | Brain research | Insects | Microscopy | Morphology | Mutation | Index Medicus
Journal Article
Nature Cell Biology, ISSN 1465-7392, 03/2010, Volume 12, Issue 3, pp. 213 - 223
Impaired selective turnover of p62 by autophagy causes severe liver injury accompanied by the formation of p62-positive inclusions and upregulation of... 
OXIDATIVE STRESS | PROTEIN | MECHANISM | CUL3-BASED E3 LIGASE | STRUCTURAL BASIS | DLG MOTIFS | DEGRADATION | MICE | BETA-CELL MASS | INDUCTION | CELL BIOLOGY | Adaptor Proteins, Signal Transducing - chemistry | Liver - pathology | Microtubule-Associated Proteins - genetics | Cytoskeletal Proteins - genetics | Gene Expression - genetics | Protein Interaction Domains and Motifs - physiology | Sequestosome-1 Protein | Humans | Oxidative Stress - physiology | Crystallography, X-Ray | Hepatocytes - pathology | Autophagy - physiology | Intracellular Signaling Peptides and Proteins - metabolism | Hepatocytes - metabolism | Liver - physiopathology | Heat-Shock Proteins - genetics | Mutation - physiology | Transfection | Organ Size - genetics | Cytoskeletal Proteins - metabolism | NF-E2-Related Factor 2 - genetics | Inclusion Bodies - metabolism | Kelch-Like ECH-Associated Protein 1 | Cell Line | Binding, Competitive - physiology | Heat-Shock Proteins - metabolism | Liver - metabolism | Models, Molecular | Mice, Transgenic | Cytoskeletal Proteins - chemistry | Mice, Knockout | Protein Interaction Mapping | Autophagy-Related Protein 7 | Animals | Models, Biological | NF-E2-Related Factor 2 - metabolism | Adaptor Proteins, Signal Transducing - genetics | Calorimetry | Signal Transduction - physiology | Mice | Adaptor Proteins, Signal Transducing - metabolism | Heat-Shock Proteins - chemistry | Protein Binding - physiology | Autophagy (Cytology) | Care and treatment | Transcription factors | Liver diseases | Physiological aspects | Genetic aspects | Research | Index Medicus
Journal Article
Science, ISSN 0036-8075, 8/2010, Volume 329, Issue 5993, pp. 805 - 810
Therapeutic efforts to restore biosynthetic processing of the cystic fibrosis transmembrane conductance regulator lacking the F508 residue (ΔF508CFTR) are... 
Cellular metabolism | RESEARCH ARTICLES | Internalization | Small interfering RNA | HeLa cells | Immunoblotting | Cystic fibrosis | Cell membranes | Biosynthesis | Recycling | Physiological regulation | TRANSMEMBRANE CONDUCTANCE REGULATOR | CFTR-DELTA-F508 | CELLS | CHIP | RETICULUM-ASSOCIATED DEGRADATION | MULTIDISCIPLINARY SCIENCES | ENDOPLASMIC-RETICULUM | CYSTIC-FIBROSIS | HSC70 | E3 UBIQUITIN LIGASE | SECRETORY PATHWAY | Temperature | Molecular Chaperones - metabolism | HSC70 Heat-Shock Proteins - metabolism | Humans | Homeostasis | Ubiquitination | Lysosomes - metabolism | Cell Membrane - metabolism | Protein Stability | Ubiquitinated Proteins - metabolism | Recombinant Proteins - metabolism | HSC70 Heat-Shock Proteins - genetics | Ubiquitinated Proteins - chemistry | Ubiquitin-Protein Ligases - metabolism | Mutant Proteins - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Glycosylation | Cystic Fibrosis Transmembrane Conductance Regulator - chemistry | Protein Folding | Protein Transport | Mutant Proteins - chemistry | HSP90 Heat-Shock Proteins - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Protein Conformation | RNA, Small Interfering | HeLa Cells | Ubiquitin-Protein Ligases - genetics | Physiological aspects | Research | Membrane proteins | Proteins | Plasma | Membranes | Cellular biology | Index Medicus | Degradation | Residues | Regulators | Quality control | Conductance | Endoplasmic reticulum
Journal Article
Nature Cell Biology, ISSN 1465-7392, 09/2010, Volume 12, Issue 9, pp. 863 - 875
Accumulation of unwanted/misfolded proteins in aggregates has been observed in airways of patients with cystic fibrosis (CF), a life-threatening genetic... 
APOPTOSIS | UNFOLDED PROTEIN RESPONSE | BECLIN 1 | DOWN-REGULATION | ENDOPLASMIC-RETICULUM | MICE | GENE-TRANSFER | TISSUE TRANSGLUTAMINASE | NEURODEGENERATIVE DISEASES | CELL-DEATH | CELL BIOLOGY | Reactive Oxygen Species - metabolism | Respiratory Mucosa - drug effects | Sequestosome-1 Protein | Humans | Transglutaminases - genetics | Phosphatidylinositol 3-Kinases - antagonists & inhibitors | Autophagy - drug effects | Young Adult | Cystamine - therapeutic use | Inflammation - metabolism | Autophagy - genetics | Nasal Polyps - metabolism | Mice, Inbred CFTR | Respiratory Mucosa - cytology | Cystic Fibrosis - metabolism | Membrane Proteins - genetics | Mice, Transgenic | Protein Transport - genetics | Apoptosis Regulatory Proteins - metabolism | Reactive Oxygen Species - antagonists & inhibitors | Models, Biological | Cystic Fibrosis - genetics | Transglutaminases - metabolism | Adolescent | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Mice | GTP-Binding Proteins | Cystic Fibrosis - drug therapy | Protein Binding - physiology | Microtubule-Associated Proteins - metabolism | Phosphatidylinositol 3-Kinases - metabolism | Salicylates - pharmacology | Epithelial Sodium Channels - genetics | Apoptosis Regulatory Proteins - genetics | Adult | Cystamine - pharmacology | Membrane Proteins - metabolism | Acetylcysteine - therapeutic use | Beclin-1 | Cystic Fibrosis Transmembrane Conductance Regulator - antagonists & inhibitors | Cell Line | Small Ubiquitin-Related Modifier Proteins - metabolism | Heat-Shock Proteins - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Antioxidants - pharmacology | Mice, Inbred Strains | Nasal Polyps - drug therapy | Antioxidants - therapeutic use | Animals | Acetylcysteine - pharmacology | Adaptor Proteins, Signal Transducing - genetics | Respiratory Mucosa - metabolism | Adaptor Proteins, Signal Transducing - metabolism | Organometallic Compounds - pharmacology | Complications and side effects | Active oxygen | Gene mutations | Lung diseases | Physiological aspects | Cystic fibrosis | Genetic aspects | Research | Health aspects | Risk factors | Index Medicus
Journal Article