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https://www.readbyqxmd.com/read/23754400/double-deletion-of-melanocortin-4-receptors-and-sapap3-corrects-compulsive-behavior-and-obesity-in-mice
#1
Pin Xu, Brad A Grueter, Jeremiah K Britt, Latisha McDaniel, Paula J Huntington, Rachel Hodge, Stephanie Tran, Brittany L Mason, Charlotte Lee, Linh Vong, Bradford B Lowell, Robert C Malenka, Michael Lutter, Andrew A Pieper
Compulsive behavior is a debilitating clinical feature of many forms of neuropsychiatric disease, including Tourette syndrome, obsessive-compulsive spectrum disorders, eating disorders, and autism. Although several studies link striatal dysfunction to compulsivity, the pathophysiology remains poorly understood. Here, we show that both constitutive and induced genetic deletion of the gene encoding the melanocortin 4 receptor (MC4R), as well as pharmacologic inhibition of MC4R signaling, normalize compulsive grooming and striatal electrophysiologic impairments in synapse-associated protein 90/postsynaptic density protein 95-associated protein 3 (SAPAP3)-null mice, a model of human obsessive-compulsive disorder...
June 25, 2013: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/23533486/aqueous-extract-of-paeonia-lactiflora-and-paeoniflorin-as-aggregation-reducers-targeting-chaperones-in-cell-models-of-spinocerebellar-ataxia-3
#2
Kuo-Hsuan Chang, Wan-Ling Chen, Li-Ching Lee, Chih-Hsin Lin, Pin-Jui Kung, Te-Hsien Lin, Yi-Ci Wu, Yih-Ru Wu, Yi-Chun Chen, Guey-Jen Lee-Chen, Chiung-Mei Chen
Spinocerebellar ataxia (SCA) types 1, 2, 3, 6, 7, and 17 as well as Huntington's disease are a group of neurodegenerative disorders caused by expanded CAG repeats encoding a long polyglutamine (polyQ) tract in the respective proteins. Evidence has shown that the accumulation of intranuclear and cytoplasmic misfolded polyQ proteins leads to apoptosis and cell death. Thus suppression of aggregate formation is expected to inhibit a wide range of downstream pathogenic events in polyQ diseases. In this study, we established a high-throughput aggregation screening system using 293 ATXN3/Q75-GFP cells and applied this system to test the aqueous extract of Paeonia lactiflora (P...
2013: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/23027934/neuroprotective-efficacy-of-aminopropyl-carbazoles-in-a-mouse-model-of-parkinson-disease
#3
Héctor De Jesús-Cortés, Pin Xu, Jordan Drawbridge, Sandi Jo Estill, Paula Huntington, Stephanie Tran, Jeremiah Britt, Rachel Tesla, Lorraine Morlock, Jacinth Naidoo, Lisa M Melito, Gelin Wang, Noelle S Williams, Joseph M Ready, Steven L McKnight, Andrew A Pieper
We previously reported the discovery of P7C3, an aminopropyl carbazole having proneurogenic and neuroprotective properties in newborn neural precursor cells of the dentate gyrus. Here, we provide evidence that P7C3 also protects mature neurons in brain regions outside of the hippocampus. P7C3 blocks 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-mediated cell death of dopaminergic neurons in the substantia nigra of adult mice, a model of Parkinson disease (PD). Dose-response studies show that the P7C3 analog P7C3A20 blocks cell death with even greater potency and efficacy, which parallels the relative potency and efficacy of these agents in blocking apoptosis of newborn neural precursor cells of the dentate gyrus...
October 16, 2012: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/23027932/neuroprotective-efficacy-of-aminopropyl-carbazoles-in-a-mouse-model-of-amyotrophic-lateral-sclerosis
#4
Rachel Tesla, Hamilton Parker Wolf, Pin Xu, Jordan Drawbridge, Sandi Jo Estill, Paula Huntington, Latisha McDaniel, Whitney Knobbe, Aaron Burket, Stephanie Tran, Ruth Starwalt, Lorraine Morlock, Jacinth Naidoo, Noelle S Williams, Joseph M Ready, Steven L McKnight, Andrew A Pieper
We previously reported the discovery of P7C3, an aminopropyl carbazole having proneurogenic and neuroprotective properties in newborn neural precursor cells of the hippocampal dentate gyrus. We have further found that chemicals having efficacy in this in vivo screening assay also protect dopaminergic neurons of the substantia nigra following exposure to the neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, a mouse model of Parkinson disease. Here, we provide evidence that an active analog of P7C3, known as P7C3A20, protects ventral horn spinal cord motor neurons from cell death in the G93A-SOD1 mutant mouse model of amyotrophic lateral sclerosis (ALS)...
October 16, 2012: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/22253794/amyloid-mediated-sequestration-of-essential-proteins-contributes-to-mutant-huntingtin-toxicity-in-yeast
#5
Natalia V Kochneva-Pervukhova, Alexander I Alexandrov, Michael D Ter-Avanesyan
BACKGROUND: Polyglutamine expansion is responsible for several neurodegenerative disorders, among which Huntington disease is the most well-known. Studies in the yeast model demonstrated that both aggregation and toxicity of a huntingtin (htt) protein with an expanded polyglutamine region strictly depend on the presence of the prion form of Rnq1 protein ([PIN+]), which has a glutamine/asparagine-rich domain. PRINCIPAL FINDINGS: Here, we showed that aggregation and toxicity of mutant htt depended on [PIN+] only quantitatively: the presence of [PIN+] elevated the toxicity and the levels of htt detergent-insoluble polymers...
2012: PloS One
https://www.readbyqxmd.com/read/21625618/prion-formation-and-polyglutamine-aggregation-are-controlled-by-two-classes-of-genes
#6
Anita L Manogaran, Joo Y Hong, Joan Hufana, Jens Tyedmers, Susan Lindquist, Susan W Liebman
Prions are self-perpetuating aggregated proteins that are not limited to mammalian systems but also exist in lower eukaryotes including yeast. While much work has focused around chaperones involved in prion maintenance, including Hsp104, little is known about factors involved in the appearance of prions. De novo appearance of the [PSI+] prion, which is the aggregated form of the Sup35 protein, is dramatically enhanced by transient overexpression of SUP35 in the presence of the prion form of the Rnq1 protein, [PIN+]...
May 2011: PLoS Genetics
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