منابع مشابه
Neuronal involvement in muscular atrophy
The innervation of skeletal myofibers exerts a crucial influence on the maintenance of muscle tone and normal operation. Consequently, denervated myofibers manifest atrophy, which is preceded by an increase in sarcolemma permeability. Recently, de novo expression of hemichannels (HCs) formed by connexins (Cxs) and other none selective channels, including P2X7 receptors (P2X7Rs), and transient r...
متن کاملOculopharyngeal involvement in familial neurogenic muscular atrophy.
A Japanese family with progressive spinal muscular atrophy is presented. Seven members in two generations were affected and the mode of inheritance was probably an autosomal recessive trait. A characteristic feature of this family was the presence of oculopharyngeal involvement in some of the affected members, in addition to the variable distribution of muscular atrophy among each of the affect...
متن کاملSpinal Muscular Atrophy: A Short Review Article
Spinal muscular atrophy (SMA) is a genetic disorder which affect nervous system and is characterized with progressive distal motor neuron weakness. The survival motor neuron (SMN) protein level reduces in patients with SMA. Two different genes code survival motor neuron protein in human genome. Skeletal and intercostal muscles denervation lead to weakness, hypotony, hyporeflexia, respiratory fa...
متن کاملPrefrontal involvement related to cognitive impairment in progressive muscular atrophy.
OBJECTIVE To examine brain activation patterns during verbal fluency performance in patients with progressive muscular atrophy (PMA) and amyotrophic lateral sclerosis (ALS). METHODS fMRI was used to examine the blood oxygen level-dependent response during letter and category fluency performance in 18 patients with PMA, 21 patients with ALS, and 17 healthy control subjects, matched for age and...
متن کاملType 0 spinal muscular atrophy with multisystem involvement.
BACKGROUND The classical forms of severe SMA type 0 is well recognised by Pediatricians. CASE CHARACTERISTICS A hypotonic neonate with severe respiratory distress at birth. OBSERVATIONS Homozygous absence of exons 7 of the Survival Motor Neuron I gene. OUTCOME Died 108 days after admission when respiratory support was withdrawn at the request of the parents. MESSAGE Spinal Muscular Atro...
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ژورنال
عنوان ژورنال: Frontiers in Cellular Neuroscience
سال: 2014
ISSN: 1662-5102
DOI: 10.3389/fncel.2014.00405