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Journal Article
The EMBO Journal, ISSN 0261-4189, 09/2015, Volume 34, Issue 17, pp. 2255 - 2271
Journal Article
Alzheimer's & Dementia: The Journal of the Alzheimer's Association, ISSN 1552-5260, 07/2019, Volume 15, Issue 7, pp. P1502 - P1502
Journal Article
Neuron, ISSN 0896-6273, 2010, Volume 67, Issue 2, pp. 199 - 212
Proteolytic cleavage of huntingtin (Htt) is known to be a key event in the pathogenesis of Huntington's disease (HD). Our understanding of proteolytic... 
HUMDISEASE | MOLNEURO | Humdisease | Molneuro | POLYGLUTAMINE TRACT | HUMAN BRAIN | N-TERMINAL FRAGMENTS | FOCAL CEREBRAL-ISCHEMIA | STRIATAL CELLS | MOUSE MODEL | CASPASE CLEAVAGE | POTENTIAL ROLE | NEUROSCIENCES | LENGTH MUTANT HUNTINGTIN | REDUCES TOXICITY | Gene Expression Regulation, Enzymologic - drug effects | Matrix Metalloproteinases - genetics | Humans | Peptides - genetics | Mice, Neurologic Mutants | Nerve Tissue Proteins - toxicity | Caspases - metabolism | Peptides - metabolism | Transfection - methods | Cell Death - genetics | Neurons - metabolism | Nuclear Proteins - drug effects | Cell Death - drug effects | Neurons - drug effects | Nuclear Proteins - genetics | Corpus Striatum - pathology | Disease Models, Animal | Matrix Metalloproteinases - classification | Drosophila | RNA, Small Interfering - pharmacology | Nuclear Proteins - metabolism | Mutation - genetics | Nerve Tissue Proteins - drug effects | Nerve Tissue Proteins - genetics | Nuclear Proteins - toxicity | Nerve Tissue Proteins - metabolism | Huntingtin Protein | Animals | Gene Expression Regulation, Enzymologic - genetics | RNA, Small Interfering - therapeutic use | Huntington Disease - genetics | Mice | Matrix Metalloproteinases - metabolism | Cell Line, Transformed | Huntington's chorea | Cell death | Proteases | Proteolysis | Neurons | Analysis | Proteins | Huntingtons disease | Enzymes | Insects | Toxicity | Genomes | Apoptosis | Index Medicus
Journal Article
PLoS ONE, ISSN 1932-6203, 07/2015, Volume 10, Issue 7, p. e0132376
Friedreich's ataxia (FRDA), the most common inherited ataxia in the Caucasian population, is a multisystemic disease caused by a significant decrease in the... 
FRATAXIN DEFICIENCY | IRON CHAPERONE | PATHWAY | MULTIDISCIPLINARY SCIENCES | YEAST MODEL | RESISTANCE | INCREASES | EXTENDS LIFE-SPAN | MECHANISMS | EXPRESSION | OXIDATIVE STRESS-RESPONSE | Aconitate Hydratase - metabolism | Superoxide Dismutase - genetics | Glutathione - metabolism | Longevity - drug effects | Antioxidants - metabolism | Humans | Male | Aldehydes - metabolism | Drosophila Proteins - metabolism | Drosophila melanogaster - genetics | Iron-Binding Proteins - metabolism | Friedreich Ataxia - genetics | Drosophila melanogaster - metabolism | RNA Interference | Adenosine Triphosphate - metabolism | Drosophila Proteins - antagonists & inhibitors | Immunosuppressive Agents - pharmacology | Repressor Proteins - metabolism | Superoxide Dismutase - metabolism | Disease Models, Animal | Malondialdehyde - metabolism | Friedreich Ataxia - metabolism | Gene Expression | Animals, Genetically Modified | Repressor Proteins - genetics | Transcription Factors - antagonists & inhibitors | Longevity - genetics | Transcription Factors - genetics | Reverse Transcriptase Polymerase Chain Reaction | Sirolimus - pharmacology | Transcription Factors - metabolism | Motor Activity - genetics | Animals | Iron-Binding Proteins - genetics | Drosophila Proteins - genetics | Oxidative Stress - drug effects | Antioxidants | Ataxia | Rapamycin | Analysis | Drosophila | Genetic screening
Journal Article
Nature, ISSN 0028-0836, 2013, Volume 498, Issue 7454, pp. 325 - 331
Journal Article
Nature Neuroscience, ISSN 1097-6256, 05/2013, Volume 16, Issue 5, pp. 562 - 570
Huntington's disease is caused by expanded CAG repeats in HTT, conferring toxic gain of function on mutant HTT (mHTT) protein. Reducing mHTT amounts is... 
CELLS | MODIFIERS | PROTEASOMAL DEGRADATION | MOTOR | NEDD8 | IN MOUSE MODEL | NEUROPATHOLOGY | GENE HOMOLOG HDH | EXPRESSION | NEUROSCIENCES | DISEASE PROGRESSION | Physiological aspects | Genetic aspects | Research | Gene mutations | Huntington's chorea | Genomics
Journal Article
PLoS ONE, ISSN 1932-6203, 07/2016, Volume 11, Issue 7, p. e0159209
Journal Article
Neuron, ISSN 0896-6273, 10/2016, Volume 92, Issue 2, pp. 407 - 418
Journal Article
Journal Article