Proteostasis is critical for neuronal maintenance and survival, and its imbalance leads to neurodegeneration with the hallmark of protein misfolding and aggregation. Macroautophagy becomes a major route for the cleara...Proteostasis is critical for neuronal maintenance and survival, and its imbalance leads to neurodegeneration with the hallmark of protein misfolding and aggregation. Macroautophagy becomes a major route for the clearance of protein aggregates that are normally poor substrates for the proteasome, the other protein quality-control machinery. As a flux process, macroautophagy （hereafter referred to as autophagy） involves the formation of the autophagosome, a double-membrane vesicle for engulfing unwanted cellular components such as protein aggregates, and the fusion of autophagosomes with lysosomes that contain many potent proteases for final degradation.展开更多
Sound is one of the information carriers often used in animal communication. Sound produced by animals is useful not only in intraspecific communication,but also in alarm or aggression.Three basic problems in hearing ...Sound is one of the information carriers often used in animal communication. Sound produced by animals is useful not only in intraspecific communication,but also in alarm or aggression.Three basic problems in hearing should be resolved i. e. frequency selectivity, pattern recognition and sound direction. With intracellular recording and single cell staining techniques, frequency selectivity of primary auditory neurons in the bushcricket has been studied. Each neuron has its characteristic best frequency (BF) and tuning curve in response to sound. The central projections of their axons in the prothoracic ganglion are unikteral, non - transsegmental and of some corresponding relation to their BFs. Neural mechanisms for frequency analysis in species - specific song recognition of the bushcricket are discussed.展开更多
Huntington＇s disease （HD） is one of the most common dominantly-inherited neurodegenerative disorders and is caused by a CAG repeat expansion in the huntingtin gene. HD is characterized by selective degeneration of ...Huntington＇s disease （HD） is one of the most common dominantly-inherited neurodegenerative disorders and is caused by a CAG repeat expansion in the huntingtin gene. HD is characterized by selective degeneration of subpopulations of neurons in the brain, however the precise underlying mechanisms how a ubiquitously expressed disease protein could target specific types of neurons for degeneration remains a critical, yet unanswered question for HD and other major neurodegenerative disorders. In this review, we describe the expanding view of selective neuronal vulnerability in HD, based on recent neuropathological and neuroimaging studies. We will also summarize the systematic effort to define the cell types in which mutant Huntingtin expression is critical for pathogenesis of vulnerable neurons in the striatum and cortex. Finally, we will describe selected, emerging molecular mechanisms that are implicated in selective disease processes in HD. Together, the field has begun to appreciate the distinct molecular pathogenic roles of mutant huntingtin in different cell types that may contribute to the selective neuronal vulnerability, with dissection of such mechanisms likely to yield novel molecular targets for HD therapy.展开更多
文摘Proteostasis is critical for neuronal maintenance and survival, and its imbalance leads to neurodegeneration with the hallmark of protein misfolding and aggregation. Macroautophagy becomes a major route for the clearance of protein aggregates that are normally poor substrates for the proteasome, the other protein quality-control machinery. As a flux process, macroautophagy （hereafter referred to as autophagy） involves the formation of the autophagosome, a double-membrane vesicle for engulfing unwanted cellular components such as protein aggregates, and the fusion of autophagosomes with lysosomes that contain many potent proteases for final degradation.
基金The Project Supported by National Natural Science Foundation of China
文摘Sound is one of the information carriers often used in animal communication. Sound produced by animals is useful not only in intraspecific communication,but also in alarm or aggression.Three basic problems in hearing should be resolved i. e. frequency selectivity, pattern recognition and sound direction. With intracellular recording and single cell staining techniques, frequency selectivity of primary auditory neurons in the bushcricket has been studied. Each neuron has its characteristic best frequency (BF) and tuning curve in response to sound. The central projections of their axons in the prothoracic ganglion are unikteral, non - transsegmental and of some corresponding relation to their BFs. Neural mechanisms for frequency analysis in species - specific song recognition of the bushcricket are discussed.
文摘Huntington＇s disease （HD） is one of the most common dominantly-inherited neurodegenerative disorders and is caused by a CAG repeat expansion in the huntingtin gene. HD is characterized by selective degeneration of subpopulations of neurons in the brain, however the precise underlying mechanisms how a ubiquitously expressed disease protein could target specific types of neurons for degeneration remains a critical, yet unanswered question for HD and other major neurodegenerative disorders. In this review, we describe the expanding view of selective neuronal vulnerability in HD, based on recent neuropathological and neuroimaging studies. We will also summarize the systematic effort to define the cell types in which mutant Huntingtin expression is critical for pathogenesis of vulnerable neurons in the striatum and cortex. Finally, we will describe selected, emerging molecular mechanisms that are implicated in selective disease processes in HD. Together, the field has begun to appreciate the distinct molecular pathogenic roles of mutant huntingtin in different cell types that may contribute to the selective neuronal vulnerability, with dissection of such mechanisms likely to yield novel molecular targets for HD therapy.
