Search results for "Chen"
showing 10 items of 702 documents
Memory deficit of children with Duchenne muscular dystrophy
2007
Several studies on intelligence of children suffering from Duchenne muscular dystrophy (DMD) found impairments in linguistic functions and memory. Memory deficit mainly involves long-term memory; short-term memory impairment was also present, even if less frequently. Some studies tried to establish a correlation between the distal deletion of the dystrophin gene and low intelligence levels. The present study investigated the neuropsychological performance in a group of DMD children (26 subjects); neuropsychological tests included: Wechsler intelligence scale for children-revised; Raven’s progressive matrices test; Rey’s complex figure test; memory and digit span tests; Benton’s visual reten…
Early alterations of the behavioural structure of mice affected by Duchenne muscular dystrophy and tested in open-field
2020
Present study has been carried out to assess whether early alterations of the behavioural structure may be detected in mice affected by Duchenne muscular dystrophy (DMD). To this purpose, both quantitative and T-pattern analysis (TPA) were used to analyse the behaviour of two groups of male, two months old mice, 18 MDX and 18 normal as control, tested in an open-field apparatus. T-pattern analysis is a multivariate technique able to reveal hidden structural features of behaviour and, in particular, its temporal characteristics. As to quantitative analyses, mean durations evidenced a significant increase of Walking, Modified Climbing and Rearing and a significant reduction of Immobile-Sniffi…
MMP-10 Is Required for Efficient Muscle Regeneration in Mouse Models of Injury and Muscular Dystrophy
2013
Abstract Matrix metalloproteinases (MMPs), a family of endopeptidases that are involved in the degradation of extracellular matrix components, have been implicated in skeletal muscle regeneration. Among the MMPs, MMP-2 and MMP-9 are upregulated in Duchenne muscular dystrophy (DMD), a fatal X-linked muscle disorder. However, inhibition or overexpression of specific MMPs in a mouse model of DMD (mdx) has yielded mixed results regarding disease progression, depending on the MMP studied. Here, we have examined the role of MMP-10 in muscle regeneration during injury and muscular dystrophy. We found that skeletal muscle increases MMP-10 protein expression in response to damage (notexin) or diseas…
Long-Term Ventilation in Neuromuscular Patients: Review of Concerns, Beliefs, and Ethical Dilemmas
2019
<b><i>Background:</i></b> Noninvasive mechanical ventilation (NIV) is an effective treatment in patients with neuromuscular diseases (NMD) to improve symptoms, quality of life, and survival. <b><i>Summary:</i></b> NIV should be used early in the course of respiratory muscle involvement in NMD patients and its requirements may increase over time. Therefore, training on technical equipment at home and advice on problem solving are warranted. Remote monitoring of ventilator parameters using built-in ventilator software is recommended. Telemedicine may be helpful in reducing hospital admissions. Anticipatory planning and palliative care should be …
Myogenic NOS and endogenous NO production are defective in colon from dystrophic (mdx) mice
2001
The aim of the present study was to evaluate whether alterations in the distribution and/or function of nitric oxide synthase (NOS) could be involved in the development of the spontaneous mechanical tone observed in colon from dystrophic (mdx) mice. By recording the intraluminal pressure of isolated colon from normal mice, we showed that Nω-nitro-L-arginine methyl ester (L-NAME) increased the tone, even in the presence of tetrodotoxin. The effect was prevented by L-arginine, nifedipine, or Ca2+-free solution. In colon from mdx mice, L-NAME was ineffective. Immunohistochemistry revealed that the presence and distribution of neuronal (nNOS), endothelial, and inducible NOS isoforms in smooth m…
An attempt to enhance neurogenesis of mdx mice via aerobic exercise and myostatin inhibition
2013
Duchennen lihasdystrofia (DMD) on perinnöllinen sairaus, jonka esiintyvyys on noin 1/3600 poikavauvasta. Siihen liittyy lihasten heikkoutta, rappeutumista ja kognitiivista vajavaisuutta. Taudin aiheuttaa mutatoitunut geeni dystrophiini proteiinille. On esitetty, että kognitiivinen vajavaisuus johtuu taudin vaikutuksesta ehkäistä neurogeneesiä. Neurogeneesi on prosessi, joka jatkuvasti synnyttää uusia hermosoluja pääasiallisesti subventikulaari alueella ja hippokampuksen dentate gyruksella. Hoitoa ja parannusta tautiin tutkitaan yleensä mdx-hiirillä, joiden taudin etiologia on riittävän lähellä ihmisten tautia. Tämä tutkimus keskittyy pyrkimykseen vaikuttaa mdx-hiirten neurogeneesiin aerobis…
Ultrastructural changes in the Interstitials Cells of Cajal and gastric dysrhythmias in mice lacking full-length dystrophin (mdx mice)
2004
At least two populations of c-kit positive interstitial cells of Cajal (ICC) lie in the gastric wall, one located at the myenteric plexus level has a pace-making function and the other located intramuscularly is intermediary in the neurotransmission and regenerates the slow waves. Both of these ICC sub-types express full-length dystrophin. Mdx mice, an animal model lacking in full-length dystrophin and used to study Duchenne muscular dystrophy (DMD), show gastric dismotilities. The aim of the present study was to verify in mdx mice whether: (i) gastric ICC undergo morphological changes, through immunohistochemical and ultrastructural analyses; and (ii) there are alterations in the electrica…
Working Memory Alterations Plays an Essential Role in Developing Global Neuropsychological Impairment in Duchenne Muscular Dystrophy
2021
BackgroundNeuropsychological profile of Indian Duchenne muscular dystrophy (DMD) subjects remains unidentified and needs to be evaluated.MethodsA total of 69 DMD and 66 controls were subjected to detailed intelligence and neuropsychological assessment. The factor indexes were derived from various components of Malin’s Intelligence Scale for Indian Children (MISIC) and Rey Auditory Verbal Learning Test (RAVLT).ResultsPoor verbal and visual memory profiles were demonstrated by DMDs, which include RAVLT-immediate recall (IR) (p = 0.042), RAVLT-delayed recall (DR) (p = 0.009), Rey–Osterrieth complex figure test (RCFT)-IR (p = 0.001), and RCFT-DR (p = 0.001). RAVLT-memory efficiency index demons…
Psychological aspects in children affected by duchenne de boulogne muscular dystrophy.
2012
Impairment of intelligence in Duchenne muscular dystrophy (DMD) patients was described by Duchenne de Boulogne himself in 1868. Further studies report intelligence disorders with mayor impairment of memory. The aim of the present study was to assess the presence of affective and personality disorders in a group of children affected by DMD. Twenty six male DMD patients, mean age eleven and four months years old, were assessed for their affective and personality disorder. Only eight subjects had a total IQ below average with major difficulties in verbal and visual-spatial memory, comprehension, arithmetic and vocabulary. All the subjects presented some disorders: tendency to marginalization a…
Areas of endemism in the Antarctic - a case study of the benthic hydrozoan genusOswaldella(Cnidaria, Kirchenpaueriidae)
2010
Aim The aim of this study is to investigate areas of endemism within the distribution of Oswaldella species in the Southern Ocean, thereby testing previous hypotheses and proposing alternative scenarios for Antarctic evolution. Location Southern Ocean, Antarctic and sub-Antarctic waters of southern South America. Methods We prepared a database for the 31 currently known species of the Antarctic genus Oswaldella, which includes geographical locations gathered from published taxonomic studies as well as materials from museums and expeditions. A parsimony analysis of endemicity (PAE) was used to test hypotheses of distribution patterns. Results Four areas of endemism are hypothesized: southern…