premotor cortex, especially the classic upper motor neuron (UMN) Betz cell population; their axons throughout the hemispheric white matter, internal capsule, pyramids, and ventrolateral spinal tracts; and the lower motor neuron (LMN) pools of the brain stem motor nuclei and the spinal anterior ...
The ability of exosomes to promote the spread of disease is also thought to play a role in neurodegenerative disorders. A common feature of these disorders is the deposition of misfolded, aggregated forms of specific proteins in defined neuroanatomical locations. As the diseases progress, these misfo...
Amyotrophic lateral sclerosis (ALS), another form of motor neuron degeneration, is the third most common neurodegenerative cause of adult death, after Alzheimer's disease and Parkinson's disease, and is significantly more common than multiple sclerosis (Motor Neurone Disease Association 1998). ...
The idea of “diseased microglia” represents a recent conceptual development in neuropathology that was first raised in the context of Creutzfeldt-Jakob disease [1].Diseasedmicroglia are different fromactivatedmicroglia, in that they are incapacitated cells whereas activated microglia are cells capably re...
To explore cell-type-specific and disease-related changes in C9 ALI-COs, we examined differentially expressed genes (DEGs) in the scRNA-seq dataset for each cluster between the C9-L1 and H-L1/H-L2 control ALI-COs. Astroglia and DLNs displayed the highest amount of DEGs, 179 and 316, re...
Novarino, G.et al. Exome sequencing links corticospinal motor neuron disease to common neurodegenerative disorders.Science (80-.).343, 506–511 (2014). ArticleADSCASGoogle Scholar Solowska, J. M. & Baas, P. W. Hereditary spastic paraplegia SPG4: what is known and not known about the dis...
At 6 mpi, pTDP-43 pathology invaded into bilateral motor cortex, pyramidal tract, lower motor neuron (LMN), and corresponding motor regulatory nucleus in the two groups of TDP-43 PFFs-injected mice. Parallelly, TDP-43 PFFs-injected mice developed ALS-like syndromes, including motor dysfunction...
(2010) miRNA malfunction causes spinal motor neuron disease. Proc Natl Acad Sci U S A 107: 13111–13116. [59] Hu YY, He SS, Wang XC, Duan QH, Khatoon S, et al. (2002) Elevated levels of phosphorylated neurofilament proteins in cerebrospinal fluid of Alzheimer disease patients. Neurosci...
Synuclein, a component of LBs, is the defining characteristic of Parkinson's disease (PD). Here, we characterize dopamine (DA) neuron鈥搒pecific autophagy-deficient mice and providein vivoevidence for LB formation. Synuclein deposition is preceded by p62 and resulted in the formation of ...
Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease. Lancet Neurol. 2011;10:253–63. Article CAS PubMed Google Scholar Johann S, Heitzer M, Kanagaratnam M, Goswami A, Rizo T, Weis J, et al. NLRP3 inflammasome is expressed by astrocytes in ...