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Mostrando 541 - 560 Resultados de 1,213 Para Buscar '"motor neurone disease"', tiempo de consulta: 2.48s Limitar resultados
  1. 541
    “…In this study, we examine the validity and reliability of the Persian version of Motor Neuron Disease Behavioral instrument (MiND‐B) questionnaire to investigate behavioral changes in Persian‐speaking ALS patients. …”
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    Examining the validity and reliability of the Persian version of the MiND‐B questionnaire in ALS patients
  2. 542
    “…Taken together, these results suggest that IF inclusions containing peripherin may play a contributory role in motor neuron disease.…”
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    Late Onset Death of Motor Neurons in Mice Overexpressing Wild-Type Peripherin
  3. 543
    “…Finally, we collate the current data on CHMP2B missense mutations, which have been reported in FTD and motor neuron disease.…”
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    Frontotemporal Dementia Caused by CHMP2B Mutations
  4. 544
    “…This review article is focused on the research progress made utilizing the wobbler mouse as animal model for human motor neuron diseases, especially the amyotrophic lateral sclerosis (ALS). …”
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    The wobbler mouse, an ALS animal model
  5. 545
    “…Collectively, its pharmacological properties designate olesoxime as a promising drug candidate for motor neuron diseases.…”
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    Olesoxime (TRO19622): A Novel Mitochondrial-Targeted Neuroprotective Compound
  6. 546
    “…Although SMA is primarily considered as a motor neuron disease, the importance of muscle defects in its pathogenesis has not been fully examined. …”
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    Myogenic program dysregulation is contributory to disease pathogenesis in spinal muscular atrophy
  7. 547
    “…Primary lateral sclerosis (PLS) is one of the forms of motor neuron disease (MND), affecting only upper motor neurons. …”
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    Symptoms of degeneration of the pyramidal tracts in conventional magnetic resonance imaging and diffusion tensor imaging in a young woman with primary lateral sclerosis
  8. 548
    “…This branch-specific disassembly of the cytoskeleton appears to be mediated by the microtubule-severing enzyme spastin, which is dysfunctional in some forms of upper motor neuron disease. Our results demonstrate a physiological role for a neurodegeneration-associated modulator of the cytoskeleton, reveal unexpected cell biology of branch-specific axon plasticity and underscore the mechanistic similarities of axon loss in development and disease.…”
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    Branch-Specific Microtubule Destabilization Mediates Axon Branch Loss during Neuromuscular Synapse Elimination
  9. 549
    “…Recent findings have suggested an important role for miRNAs in the pathogenesis of motor neuron diseases, including SMA. Motor neuron‐specific miRNA dysregulation in SMA might be implicated in their selective vulnerability. …”
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    miRNA in spinal muscular atrophy pathogenesis and therapy
  10. 550
    “…Motor neuron diseases (MNDs) are a debilitating subset of diseases, which result in progressive neuronal destruction and eventual loss of voluntary muscular function. …”
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    Amyotrophic Lateral Sclerosis and its Mimics/Variants: A Comprehensive Review
  11. 551
    “…Amyotrophic lateral sclerosis and frontotemporal lobar degeneration are incurable motor neuron diseases associated with muscle weakness, paralysis and respiratory failure. …”
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    RNA as a key factor in driving or preventing self-assembly of the TAR DNA-binding protein 43
  12. 552
    por Guo, Wenting, Van Den Bosch, Ludo
    Publicado 2017
    “…Amyotrophic lateral sclerosis (ALS) is the most common fatal motor neuron disease in adults and no effective treatments exist. …”
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    Therapeutic potential of HDAC6 in amyotrophic lateral sclerosis
  13. 553
    “…Oculomotor neurons, which regulate eye movement, are resilient to degeneration in the lethal motor neuron disease amyotrophic lateral sclerosis (ALS). …”
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    Modeling Motor Neuron Resilience in ALS Using Stem Cells
  14. 554
    “…Unsurprisingly, mutations in dynein are causative of various developmental neuropathies and motor neuron diseases. These ‘dyneinopathies’ define a broad spectrum of diseases with no known correlation between mutation identity and disease state. …”
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    Molecular basis for dyneinopathies reveals insight into dynein regulation and dysfunction
  15. 555
    “…Thus, the classical complement pathway plays critical roles in the refinement of developing motor circuits, while its aberrant activation contributes to motor neuron disease.…”
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    The Classical Complement Pathway Mediates Microglia-Dependent Remodeling of Spinal Motor Circuits during Development and in SMA
  16. 556
    por Kristensson, Krister
    Publicado 1992
    “…Owing to their selectivity in attack on regions of nervous tissue, governed by viral factors and by routes of invasion, viral receptors or metabolic machineries of infected cells, certain viral infections show similarities in distribution of their resulting lesions in the nervous system to that of the common human neurodegenerative diseases (namely, motor neurons disease, Parkinson’s disease, and Alzheimer’s disease). …”
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    Potential role of viruses in neurodegeneration
  17. 557
    “…Konzo, a distinct upper motor neuron disease associated with a cyanogenic diet and chronic malnutrition, predominately affects children and women of childbearing age in sub-Saharan Africa. …”
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    The gut microbiome in konzo
  18. 558
    “…Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a progressive neurodegenerative disease characterized by the weakness of voluntary muscles due to the loss of motor neurons. …”
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    Ethnical Disparities in Response to Edaravone in Patients With Amyotrophic Lateral Sclerosis
  19. 559
    “…Causal mutations in SPTLC1 (serine palmitoyltransferase long chain subunit 1) gene within the lipogenic pathway have been identified in amyotrophic lateral sclerosis (ALS), a paralytic and fatal motor neuron disease. Furthermore, lipid dysmetabolism within the central nervous system and circulation is associated with ALS. …”
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    Deciphering lipid dysregulation in ALS: from mechanisms to translational medicine
  20. 560
    “…There is increasing genetic evidence for the role of microglia in neurodegenerative diseases, including Alzheimer’s, Parkinson’s, and motor neuron disease. Therefore, there is a need to generate authentic in vitro models to study human microglial physiology. …”
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    Single-cell transcriptomics defines an improved, validated monoculture protocol for differentiation of human iPSC to microglia
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