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Gene therapy ameliorates bowel dysmotility and enteric neuron degeneration and extends survival in lysosomal storage disorder mouse models.
Sci Transl Med
January 2025
Department of Pediatrics, Washington University in St. Louis, School of Medicine, St. Louis, MO 63110, USA.
Children with neurodegenerative disease often have debilitating gastrointestinal symptoms. We hypothesized that this may be due at least in part to underappreciated degeneration of neurons in the enteric nervous system (ENS), the master regulator of bowel function. To test this hypothesis, we evaluated mouse models of neuronal ceroid lipofuscinosis type 1 and 2 (CLN1 and CLN2 disease, respectively), neurodegenerative lysosomal storage disorders caused by deficiencies in palmitoyl protein thioesterase-1 and tripeptidyl peptidase-1, respectively.
View Article and Find Full Text PDFFunctional and Structural Investigation of Myenteric Neurons in the Human Colon.
Gastro Hep Adv
August 2024
Institute for Physiology and Cell Biology, University of Veterinary Medicine Hannover, Foundation, Hannover, Germany.
Background And Aims: The enteric nervous system independently controls gastrointestinal function including motility, which is primarily mediated by the myenteric plexus, therefore also playing a crucial role in functional intestinal disorders. Live recordings from human myenteric neurons proved to be challenging due to technical difficulties. Using the neuroimaging technique, we are able to record human colonic myenteric neuronal activity and investigate their functional properties in a large cohort of patients.
View Article and Find Full Text PDFGut-derived lactic acid enhances tryptophan to 5-hydroxytryptamine in regulation of anxiety via .
Gut Microbes
December 2025
MOE/NHC/CAMS Key Lab of Medical Molecular Virology, School of Basic Medical Sciences, & National Clinical Research Center for Aging and Medicine, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
The gut microbiota plays a pivotal role in anxiety regulation through pathways involving neurotransmitter production, immune signaling, and metabolic interactions. Among these, gut-derived serotonin (5-hydroxytryptamine, 5-HT), synthesized from tryptophan metabolism, has been identified as a key mediator. However, it remains unclear whether specific microbial factors regulate tryptophan metabolism to influence 5-HT production and anxiety regulation.
View Article and Find Full Text PDFGut Neuropathies and Intestinal Motility Disorders.
Neurogastroenterol Motil
January 2025
College of Medicine and Public Health, Flinders University, Adelaide, South Australia, Australia.
Background: The enteric nervous system plays a key role in the coordination of gastrointestinal motility together with sympathetic, parasympathetic, and extrinsic sensory pathways. In some cases, abnormalities in neural activity in these pathways contribute to disorders of gut motility. Where this is associated with damage or death of enteric neurons, usually detected by microscopy, this is considered a gut neuropathy.
View Article and Find Full Text PDFDistribution and neurochemical characterisation of neurons containing neuregulin 1 in the enteric nervous system within the porcine small intestine.
J Vet Res
December 2024
Institute of Biology, College of Natural Sciences, University of Rzeszów, 35-310 Rzeszów, Poland.
Introduction: The enteric nervous system (ENS) in the wall of the gastrointestinal tract is complex and comprises many neurons, which are differentiated in terms of structure, function and neurochemistry. Neuregulin 1 (NRG 1) is one of the neuronal factors synthesised in the ENS about the distribution and functions of which relatively little is known. The present study is the first description of the distribution of NRG 1 in the ENS in various segments of the porcine small intestine.
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