Characterization of Gastric Mucosa Biopsies Reveals Alterations in HuntingtonTMs Disease Œ PLOS Currents Huntington Disease
نویسندگان
چکیده
Weight loss is an important complication of Huntington’s disease (HD), however the mechanism for weight loss in HD is not entirely understood. Mutant huntingtin is expressed in the gastrointestinal (GI) tract and, in HD mice, mutant huntingtin inclusions are found within the enteric nervous system along the GI tract. A reduction of neuropeptides, decreased mucosal thickness and villus length, as well as gut motility impairment, have also been shown in HD mice. We therefore set out to study gastric mucosa of patients with HD, looking for abnormalities of mucosal cells using immunohistochemistry. In order to investigate possible histological differences related to gastric acid production, we evaluated the cell density of acid producing parietal cells, as well as gastrin producing cells (the endocrine cell controlling parietal cell function). In addition, we looked at chief cells and somatostatin-containing cells. In gastric mucosa from HD subjects, compared to control subject biopsies, a reduced expression of gastrin (a marker of G cells) was found. This is in line with previous HD mouse studies showing reduction of GI tract neuropeptides.
منابع مشابه
Characterization of Gastric Mucosa Biopsies Reveals Alterations in Huntington's Disease
Weight loss is an important complication of Huntington's disease (HD), however the mechanism for weight loss in HD is not entirely understood. Mutant huntingtin is expressed in the gastrointestinal (GI) tract and, in HD mice, mutant huntingtin inclusions are found within the enteric nervous system along the GI tract. A reduction of neuropeptides, decreased mucosal thickness and villus length, a...
متن کاملCurrent Pharmacological Management in Juvenile HuntingtonTMs Disease Œ PLOS Currents Huntington Disease
Background: The clinical presentation of Juvenile Huntington’s Disease (JHD) can be very different from adult-onset HD with little evidence to guide symptomatic management. Aim: To survey the current use of pharmacological treatments for JHD. Methods: Patients were identified through the HD Association, Hospital Doctors and the European Huntington’s Disease Network REGISTRY study. Results: The ...
متن کاملA mixed fixed ratio/progressive ratio procedure reveals an apathy phenotype in the BAC HD and the z_Q175 KI mouse models of HuntingtonTMs disease Œ PLOS Currents Huntington Disease
Apathy, characterized by generally reduced interest in and likelihood to perform goal-directed actions, is a recognized symptom of Huntington’s disease (HD), a devastating neurological disorder caused by a CAG repeat expansion of the Htt gene located on chromosome 4. The present experiments used a modified progressive ratio task that incorporated a fixed-ratio schedule of reinforcement componen...
متن کاملCharacterization of Striatal Neuronal Loss and Atrophy in the R6/2 Mouse Model of HuntingtonTMs Disease Œ PLOS Currents Huntington Disease
Striatal neuronal degeneration and loss is an important feature of human Huntington’s disease (HD). R6/2 HD mice recapitulate many features of human HD including striatal atrophy. While striatal neuronal atrophy and loss is reported in R6/2 HD mice the degree of neuronal loss and the characteristics of cell body atrophy are unclear. We used stereological approaches to estimate whole striatal ne...
متن کاملNovel Nut and Bolt Task Quantifies Motor Deficits in Premanifest and Manifest HuntingtonTMs Disease Œ PLOS Currents Huntington Disease
Background: We investigated the use of a simple novel nut and bolt task in premanifest and manifest Huntington’s disease (HD) patients to detect and quantify motor impairments at all stages of the disease. Methods: Premanifest HD (n=24), manifest HD (n=27) and control (n=32) participants were asked to screw a nut onto a bolt in one direction, using three different sized bolts with their left an...
متن کامل