Central and peripheral defects in motor units of the diaphragm of spinal muscular atrophy mice
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- 作者：Anuja Nevea ; b ; Judith Trü ; ba ; Smita Saxenaa ; Daniel Schü ; mperlia ; daniel.schuemperli@izb.unibe.ch" class="auth_mail" title="E-mail the corresponding author
- 关键词：AIFM1 ; apoptosis inducing factor 1 ; ALS ; Amyotropic Lateral Sclerosis ; BTX-Rho ; rhodamine-conjugated α-bungarotoxin ; ChAT ; choline acetyltransferase ; FHL1 ; four and half lim domain 1 ; GlyT2 ; glycine transporter 2 ; Hsp60 ; heat shock protein 60 ; IHC ; immunohistochemistry ; MN ; motoneuron ; NDUFA9 ; nicotinamide adenine dinucleotide dehydrogenase (ubiquinone) 1 ; alpha subcomplex ; 9 ; NMJ ; neuromuscular junction ; PANTHER ; Protein Analysis Through Evolutionary Relationships ; PBS ; phosphate buffered salin
- 刊名：Molecular and Cellular Neuroscience
- 出版年：2016
- 出版时间：January 2016
- 年：2016
- 卷：70
- 期：Complete
- 页码：30-41
- 全文大小：1279 K
文摘
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We analysed aspects of diaphragm innervation in a severe mouse SMA model.
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Motoneuron cell bodies: excitatory synaptic inputs reduced, inhibitory inputs normal
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Diaphragm NMJs: secretory components and marker staining of perisynaptic Schwann cells reduced
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We demonstrate the feasibility of a proteomic analysis of diaphragm samples enriched in NMJs.
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Proteomics reveal extensive changes in mitochondrial proteins and regulatory molecules.