scholarly journals Expression of Human Mutant Huntingtin Protein in Drosophila Hemocytes Impairs Immune Responses

2019 ◽  
Vol 10 ◽  
Author(s):  
Yu-Hsien Lin ◽  
Houda Ouns Maaroufi ◽  
Emad Ibrahim ◽  
Lucie Kucerova ◽  
Michal Zurovec
Keyword(s):  
Immune Responses ◽  
Mutant Huntingtin ◽  
Huntingtin Protein ◽  
Mutant Huntingtin Protein

scholarly journals Mutant huntingtin protein alters the response of microglial cells to inflammatory stimuli

2019 ◽  
Author(s):  
David W. Donley ◽  
Ryan Nelson ◽  
Jason P. Gigley ◽  
Jonathan H. Fox
Keyword(s):  
Nitric Oxide ◽  
Immune Responses ◽  
Immune Cells ◽  
Interleukin 6 ◽  
Microglial Cells ◽  
Mutant Huntingtin ◽  
Wild Type ◽  
Huntingtin Protein ◽  
Inflammatory Stimuli ◽  
Mutant Huntingtin Protein

AbstractHuntington’s disease (HD) is a progressive neurodegenerative disease that affects the striatum and cerebral cortex. It is caused by a dominant CAG trinucleotide expansion in exon 1 of theHTTgene. Mutant huntingtin protein (mHtt) is expressed in neurons and immune cells. HD patients demonstrate altered blood cytokine profiles and altered responses of peripheral immune cells to inflammatory stimuli. However, the effects of mHtt on microglial immune responses are not fully understood. Herein we discuss the current understanding of how mHtt alters microglial inflammatory responses. Using lentivirus, we expressed the N171 N-terminal fragment of wild-type or mhtt containing 18 and 82 glutamine repeats in cultured EOC-20 microglial cells. We then measured responses to lipopolysaccharide or interleukin-6. Mutant huntingtin-expressing microglial cells produced less interleukin-6 and nitric oxide in response to lipopolysaccharide stimulation than wild-type huntingtin-expressing cells. However, mHtt-expressing microglia stimulated with interleukin-6 produced more nitric oxide than wild-type cells. Mutant huntingtin-expressing cells had higher basal NF-κB and further elevations of NF-κB after interleukin-6 but not lipopolysaccharide stimulation. Thus we demonstrate the potential of mHtt to dampen responses to lipopolysaccharide but potentiate responses to interleukin-6. This work adds to the emerging understanding that mHtt alters not only baseline status of cells but may also result in altered immune responses dependent on the nature of the inflammatory stimuli. We also present our perspective that in human HD the extent of inflammation may depend, in part, on altered responses to varied inflammatory stimuli including environmental factors such as infection.

scholarly journals The pathobiology of perturbed mutant huntingtin protein–protein interactions in Huntington's disease

2019 ◽  
Vol 151 (4) ◽  
pp. 507-519 ◽  
Author(s):  
Erich E. Wanker ◽  
Anne Ast ◽  
Franziska Schindler ◽  
Philipp Trepte ◽  
Sigrid Schnoegl
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Protein Interactions ◽  
Mutant Huntingtin ◽  
Protein Protein Interactions ◽  
Huntingtin Protein ◽  
Mutant Huntingtin Protein

scholarly journals The Aggregation-Prone Mutant Huntingtin Protein in a Cellular Context - Approaches by Super-Resolution Imaging

2014 ◽  
Vol 106 (2) ◽  
pp. 683a ◽  
Author(s):  
Steffen J. Sahl ◽  
Willianne I.M. Vonk ◽  
Lucien E. Weiss ◽  
Lana Lau ◽  
Judith Frydman ◽  
...  
Keyword(s):  
Super Resolution ◽  
Mutant Huntingtin ◽  
Huntingtin Protein ◽  
Mutant Huntingtin Protein ◽  
Cellular Context ◽  
Resolution Imaging

Distinct aggregation and cell death patterns among different types of primary neurons induced by mutant huntingtin protein

2004 ◽  
Vol 89 (4) ◽  
pp. 974-987 ◽  
Author(s):  
Kazuhiko Tagawa ◽  
Masataka Hoshino ◽  
Tomohiro Okuda ◽  
Hiroko Ueda ◽  
Hiroshi Hayashi ◽  
...  
Keyword(s):  
Cell Death ◽  
Primary Neurons ◽  
Mutant Huntingtin ◽  
Huntingtin Protein ◽  
Different Types ◽  
Mutant Huntingtin Protein

Effect of Pesticides on the Aggregation of Mutant Huntingtin Protein

2012 ◽  
Vol 45 (3) ◽  
pp. 405-414 ◽  
Author(s):  
Ruhi S. Deshmukh ◽  
Rajeev K. Chaudhary ◽  
Ipsita Roy
Keyword(s):  
Mutant Huntingtin ◽  
Huntingtin Protein ◽  
Mutant Huntingtin Protein
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