Disease relevance of par

• Together with the rapid progression and the severity of the disease, these results suggest that the paralysé mouse may represent a good animal model for studying early-onset human motor neuron diseases such as spinal muscular atrophy [1].
• The results show that subcutaneous injections of 1 or 10 mg/kg BDNF did not have any significant effect in increasing the mean survival time of mutant mice or in preventing the loss of motor function and total body weight in paralysé mice [2].
• The adult AChR is the target for autoantibodies in myasthenia gravis (MG), and antibodies that block the function of fetal AChR can cross the placenta and paralyse the developing baby causing joint contractures [3].

High impact information on par

• The paralysé mouse mutant: a new animal model of anterior horn motor neuron degeneration [1].
• We report here that at the time the mutant phenotype can be first identified (i.e. approximately PN8), lumbar motoneuron numbers in the lateral motor column of the spinal cord of paralysé mice were not significantly different from those of control littermates [1].
• Nerve and muscle development in paralysé mutant mice [4].
• The paralysé muscles reacted to experimental denervation with an increase in extrajunctional acetylcholine receptor numbers [4].
• Muscle fibers from paralysé mutants had a unimodal distribution of diameters and normal numbers and distributions of acetylcholine receptors [4].

Biological context of par

• We conclude that the major expression of the paralysé mutant phenotype is an arrest in development of both nerve and muscle during the first week after birth [4].

Anatomical context of par

• In this study, we tested BDNF on an animal model of early-onset motoneuron disease: the paralysé mouse mutant, characterized by a progressive skeletal muscle atrophy and the loss of 30-35% of spinal lumbar motoneurons between the first and second week post-natal [2].
• The mutant mouse 'paralysé' exhibits a spontaneous regression of motor nerve endings, with concomitant paralysis [5].
• The toxin can reversibly paralyse cockroaches for several hours, with an ED50 of 127 +/- 54 microg/g. HWTX-II blocks neuromuscular transmission in an isolated mouse phrenic nerve diaphragm preparation and acts cooperatively to potentiate the activity of huwentoxin-I [6].

Other interactions of par

• The weight and choline acetyltransferase activity of specific muscles and the number of motoneurons in the spinal cords were identical in BDNF-treated and placebo-injected paralysé mice [2].
• Modulation of HSP25 expression during anterior horn motor neuron degeneration in the paralysé mouse mutant [7].

Analytical, diagnostic and therapeutic context of par

• Western blot analysis reveals that the level of Hsp25 was strikingly different in paralysé as compared to control littermates [7].

References