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| 1 | Loss of CREST leads to neuroinflammatory responses and ALS-like motor defects in mice显示文摘Background:Amyotrophic lateral sclerosis(ALS)is a late onset neurodegenerative disease with fast progression.ALS has heavy genetic components in which a series of genetic mutations have been identified.In 2013,Mutations of the CREST gene(also known as SS18L1),which functions as a calcium-regulated transcriptional activator,were found in sporadic ALS patients.However,the pathogenic causality and mechanisms of ALS-associated mutations of CREST remain to be determined.Methods:In this study,we constructed CREST knockout and Q394X knock-in mice with CRISPR/Cas9 system.Using biochemical and imaging tools,we illustrated core pathological phenotypes in CREST mutant mice and claimed the possible pathogenic mechanisms.Furthermore,we also observed locomotion defects in CREST mutant mice with behavioural tests.Results:We demonstrate that ALS-related CREST-Q388X mutation exhibits loss-of-function effects.Importantly,the microglial activation was prevalent in CREST haploinsufficiency mice and Q394X mice mimicking the human CREST Q388X mutation.Furthermore,we showed that both CREST haploinsufficiency and Q394X mice displayed deficits in motor coordination.Finally,we identified the critical role of CREST-BRG1 complex in repressing the expression of immune-related cytokines including Ccl2 and Cxcl10 in neurons,via histone deacetylation,providing the molecular mechanisms underlying inflammatory responses within mice lack of CREST.Conclusion:Our findings indicate that elevated inflammatory responses in a subset of ALS may be caused by neuron-derived factors,suggesting potential therapeutic methods through inflammation pathways. | Cheng Cheng Kan Yang Xinwei Wu Yuefang Zhang Shifang Shan Aaron Gitler Anirvan Ghosh Zilong Qiu | 2019 | Translational Neurodegeneration2019，8，1： | 2 |
| 2 | Alpha-synuclein is part of a diverse and highly conserved interaction network that includes PARK9 and manganese toxicity 显示文摘 | Gitler A D Chesi A Geddie M L | 2009 | Nat Genet2009，41，3： | 1 |
| 3 | Cardiac hypertrophy and histone deacetylase-dependent transcriptional repression mediated by the atypical homeodomain protein Hop显示文摘 | Kook H Lepore J Gitler AD | 2003 | J Clin Invest2003，112，6： | 1 |
| 4 | Real time imaging of calcium-induced localized proteolytic activity after axotomy and its relation to growth cone formation显示文摘 | Gitler D Spira ME | | 0，，06： | 1 |
| 5 | Short window of opportunity for calpain induced growth cone formation after axotomy of aplysia neurons显示文摘 | Gitler D Spira ME | 2002 | J Neurobiol2002，52，4： | 1 |
| 6 | Different presynaptic roles of synapsins at excitatory and inhibitory synapses 显示文摘 | Gitler D Takagishi Y Feng J | 2004 | J Neurosci2004，24，11： | 1 |
| 7 | The tip of the iceberg:RNAbinding proteins with prion-like domains in neurodegenerative disease显示文摘 | King OD Gitler AD Shorter J | 2012 | Brain Res2012，1462，： | 1 |
| 8 | Torsade de pointes induced by erythromycin显示文摘 | Berger Ls Buffa SD | 1994 | Chest1994，105，： | 1 |
| 9 | Different presynaptic roles of synapsins at excitatory and inhibitory synapses显示文摘 | Gitler D Takagishi Y Feng J | | 0，，： | 1 |
| 10 | The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect显示文摘 | Suzana Gispert Alexander Kurz Stefan Waibel Peter Bauer Inga Liepelt Christof Geisen Aaron D. Gitler Tim Becker Markus Weber Daniela Berg Peter M. Andersen Rejko Krüger Olaf Riess Albert C. Ludolph Georg Auburger | 2011 | Neurobiology of Disease2011，，1： | 1 |
| 11 | Alpha-synuclein blocks ER-Golgi traffic and Rab1 rescues neuron loss in Parkinson's models显示文摘 | COOPER A A GITLER A D CASHIKAR A | 2006 | Science2006，313，： | 1 |
| 12 | Hop Is an Unusual Homeobox Gene that Modulates Cardiac Development显示文摘 | Fabian Chen Hyun Kook Rita Milewski Aaron D. Gitler Min Min Lu Jun Li Ronniel Nazarian Robert Schnepp Kuangyu Jen Christine Biben Greg Runke Joel P. Mackay Jiri Novotny Robert J. Schwartz Richard P. Harvey Mary C. Mullins Jonathan A. Epstein | 2002 | Cell2002，，6： | 1 |
| 13 | Regulating heart development: the role ofNfl 显示文摘 | Gitler AD Epstein JA | 2003 | Cell Cycle2003，2，2： | 1 |
| 14 | Molecular markers of cardiac endocardial cushion development显示文摘 | GITLER A D LU M M JIANG Y Q et ol | 2003 | Developmental Dynamics2003，228，4： | 1 |
| 15 | Short window of opportunity for calpain induced growth cone formation after axotomy of aplysia neurons显示文摘 | Gitler D Spira ME | 2002 | J Neuro biol2002，52，4： | 1 |
| 16 | Cardiac hypertrophy and histone deacetylase-dependent transcriptional repression mediated by the atypical homeedomain protein Hop 显示文摘 | Kook H Lepore JJ Gitler AD | 2003 | J Clin Invest2003，112，86： | 1 |
| 17 | α-Synuclein blocks ER-Golgi traffic and Rabl I resues neuron loss in Parkinson models显示文摘 | Cooper A A Gitler A D Cashikar A | 2006 | Science2006，313，： | 1 |
| 18 | Real time imagingof calcium-induced localized protrolytic activity after axotomy and its relation to growth cone formation显示文摘 | Gitler D Spira M E | 1998 | Neuron1998，20，6： | 1 |
| 19 | Cardiac hypertrophy and histone deacetylase-dependent transcriptional repression mediated by the atypical homeodomain protein Hop显示文摘 | Kook H Lepore JJ Gitler AD | 2003 | J Clin Invest2003，112，6： | 1 |
| 20 | PlexinD1 and semaphorin signaling are required in endothelial cells for cardiovascular development 显示文摘 | Gitler AD Lu MM Epstein JA | 2004 | Dev Cell2004，7，1： | 1 |
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