A Muscle Perspective on the Pathophysiology of Amyotrophic Lateral Sclerosis - Differences between extraocular and limb muscles
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چکیده
..................................................................................................................... VI ABBREVIATIONS ....................................................................................................... VIII LIST OF ORIGINAL PAPERS ..................................................................................... IX
منابع مشابه
Distinct Changes in Synaptic Protein Composition at Neuromuscular Junctions of Extraocular Muscles versus Limb Muscles of ALS Donors
The pathophysiology of amyotrophic lateral sclerosis (ALS) is very complex and still rather elusive but in recent years evidence of early involvement of the neuromuscular junctions (NMJs) has accumulated. We have recently reported that the human extraocular muscles (EOMs) are far less affected than limb muscles at the end-stage of ALS from the same donor. The present study aimed to compare the ...
متن کاملUnchanged Neurotrophic Factors and Their Receptors Correlate With Sparing in Extraocular Muscles in Amyotrophic Lateral Sclerosis.
Purpose To investigate the impact of amyotrophic lateral sclerosis (ALS) on the extraocular muscles (EOMs) by examining the distribution of neurotrophic factors (NTFs) and their receptors in EOMs and limb muscles from ALS transgenic mice. Methods Muscle samples collected from transgenic mice overexpressing human superoxide dismutase type 1 mutations (SOD1G93A, the most widely used mouse model...
متن کاملAnalysis of Neurotrophic Factors in Limb and Extraocular Muscles of Mouse Model of Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is currently an incurable fatal motor neuron syndrome characterized by progressive weakness, muscle wasting and death ensuing 3-5 years after diagnosis. Neurotrophic factors (NTFs) are known to be important in both nervous system development and maintenance. However, the attempt to translate the potential of NTFs into the therapeutic options remains limited d...
متن کاملDifferent impact of ALS on laminin isoforms in human extraocular muscles versus limb muscles.
PURPOSE To determine the impact of amyotrophic lateral sclerosis (ALS) on the extraocular muscles (EOMs) by examining the laminin isoform composition of the basement membranes (BMs) in EOMs and limb muscles from donors with ALS. METHODS Muscle samples collected at autopsy from ALS donors and from transgenic mice overexpressing human superoxide dismutase type I mutations (D90A or G93A), and ag...
متن کاملUnaffected motor endplate occupancy in eye muscles of ALS G93A mouse model.
Amyotrophic lateral sclerosis (ALS) is a progressive, lethal neurodegenerative disorder characterised by selective loss of motor neurons with accompanying muscle paralysis and respiratory failure. Despite progressive paralysis in trunk and extremity muscles, disturbed eye motility is not a hallmark of ALS. Extraocular muscles (EOMs) of terminal ALS patients show far less morphological signs of ...
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تاریخ انتشار 2016