A5054346103- Lysosomal Storage Disorders Research
- Cellular transport and secretion
- Olfactory and Sensory Function Studies
- Biochemical Analysis and Sensing Techniques
- Advanced Chemical Sensor Technologies
- Calcium signaling and nucleotide metabolism
- Neurobiology and Insect Physiology Research
- Neuroscience and Neuropharmacology Research
- Glycogen Storage Diseases and Myoclonus
- Sphingolipid Metabolism and Signaling
University of Illinois Chicago
2021-2025
Sensory systems must perform the dual and opposing tasks of being sensitive to weak stimuli while also maintaining information content in dense variable sensory landscapes. This occurs olfactory system, where OSNs are highly low concentrations odors maintain discriminability complex odor environments. How neurons (OSNs) both sensitivity sparsity is poorly understood. Here, we investigated whether calcium-activated chloride channel, TMEM16B, may support these roles male female mice. We used...
Palmitoylation and depalmitoylation represent dichotomic processes by which a labile posttranslational lipid modification regulates protein trafficking degradation. The depalmitoylating enzyme, palmitoyl-protein thioesterase 1 (PPT1), is associated with the devastating pediatric neurodegenerative condition, infantile neuronal ceroid lipofuscinosis (CLN1). CLN1 characterized accumulation of autofluorescent lysosomal storage material (AFSM) in neurons robust neuroinflammation. Converging lines...
Infantile neuronal ceroid lipofuscinosis (CLN1 Batten Disease) is a devastating pediatric lysosomal storage disease caused by pathogenic variants in the CLN1 gene, which encodes depalmitoylation enzyme, palmitoyl-protein thioesterase 1 (PPT1). patients present with visual deterioration, psychomotor dysfunction, and recurrent seizures until neurodegeneration results death, typically before fifteen years of age. Histopathological features include aggregation autofluorescent material (AFSM), as...
Abstract Infantile neuronal ceroid lipofuscinosis (CLN1 Batten Disease) is a devastating pediatric lysosomal storage disease caused by pathogenic variants in the CLN1 gene, which encodes depalmitoylation enzyme, palmitoyl-protein thioesterase 1 (PPT1). patients present with visual deterioration, psychomotor dysfunction, and recurrent seizures until neurodegeneration results death, typically before fifteen years of age. Histopathological features include aggregation autofluorescent material...
The sensory modalities are anatomically, biochemically and physiologically organized to detect perceive vanishingly small quantities of environmental stimulus energies. visual system's sensitivity is sufficient single photons, while in the olfactory system, odorants can be detected range parts per billion. To achieve such sensitivity, both systems have evolved biochemical amplifiers their respective transduction currents. Olfactory begins when volatilized odorant molecules bind receptor (OR)...
Abstract Sensory systems must perform the dual and opposing tasks of being sensitive to weak stimuli while also maintaining information content in dense variable sensory landscapes. This occurs olfactory system, where OSNs are highly low concentrations odors maintain discriminability complex odor environments. How neurons (OSNs) both sensitivity sparsity is not well understood. Here, we investigated whether calcium-activated chloride channel, TMEM16B, may support these roles OSNs. We used...
Summary Palmitoylation and depalmitoylation are the dichotomic processes of lipid modification regulating protein trafficking, recycling, degradation, thereby controlling proteostasis. Despite our understanding palmitoylation, is far less studied. Here, we study a lysosomal depalmitoylating enzyme, palmitoyl-protein thioesterase 1 (PPT1), associated with devastating neurodegenerative condition CLN1 disease show that dark-rearing Ppt1 -/- mice, which induces synaptic upscaling in vivo ,...