Distinct Etiological Roles for Myocytes and Motor Neurons in a Mouse Model of Kennedy's Disease/Spinobulbar Muscular Atrophy
نویسندگان
چکیده
منابع مشابه
Distinct and overlapping alterations in motor and sensory neurons in a mouse model of spinal muscular atrophy.
Motor neuron degeneration is the predominant pathological feature of spinal muscular atrophy (SMA). In patients with severe forms of the disease, additional sensory abnormalities have been reported. However, it is not clear whether the loss of sensory neurons is a common feature in severe forms of the disease, how many neurons are lost and how loss of sensory neurons compares with motor neuron ...
متن کاملDistinct and Overlapping Alterations in Motor and Sensory Neurons in a Mouse Model of Spinal Muscular Atrophy (SMA)
Motor neuron degeneration is the predominant pathological feature of spinal muscular atrophy (SMA). In patients with severe forms of the disease, additional sensory abnormalities have been reported. However, it is not clear whether the loss of sensory neurons is a common feature in severe forms of the disease, how many neurons are lost, and how loss of sensory neurons compares with motor neuron...
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We examined the electrophysiological activity of motor neurons from the mouse model of severe spinal muscular atrophy (SMA) using two different methods: whole cell patch clamp of neurons cultured from day 13 embryos; and multi-electrode recording of ventral horns in spinal cord slices from pups on post-natal days 5 and 6. We used the MED64 multi-electrode array to record electrophysiological ac...
متن کاملEarly Functional Impairment of Sensory-Motor Connectivity in a Mouse Model of Spinal Muscular Atrophy
To define alterations of neuronal connectivity that occur during motor neuron degeneration, we characterized the function and structure of spinal circuitry in spinal muscular atrophy (SMA) model mice. SMA motor neurons show reduced proprioceptive reflexes that correlate with decreased number and function of synapses on motor neuron somata and proximal dendrites. These abnormalities occur at an ...
متن کاملTranscriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn2b/- mouse model of spinal muscular atrophy
INTRODUCTION The term motor neuron disease encompasses a spectrum of disorders in which motor neurons are the lost. Importantly, while some motor neurons are lost early in disease and others remain intact at disease end-stage. This creates a valuable experimental paradigm to investigate the factors that regulate motor neuron vulnerability. Spinal muscular atrophy is a childhood motor neuron dis...
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ژورنال
عنوان ژورنال: Journal of Neuroscience
سال: 2015
ISSN: 0270-6474,1529-2401
DOI: 10.1523/jneurosci.3599-14.2015