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• W3080518452 endingPage "724" @default.
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• W3080518452 abstract "The human motor neuron appears uniquely susceptible to distal perturbation at the NMJ. Thus, in diseases in which motor neurons are lost, their demise may begin at the nerve terminal, a phenomenon referred to as dying back. Molecular mechanisms underpinning muscle–nerve uncoupling are only beginning to be understood but are critical to elucidate in order to obtain a better understanding of sporadic ALS, a devastating disease of unknown etiology. Peripheral interventions aimed at stabilizing the NMJ could be broadly applicable to neuromuscular disease, and synergistic with targeted, disease-specific therapies, such as SMN protein-restoring drugs for SMA. In SBMA, expression of the mutant androgen receptor protein in skeletal muscle has been established as the primary driver of lower motor neuron demise, revealing skeletal muscle as the obvious and highly accessible cellular target for future therapeutic intervention. The neuromuscular junction (NMJ) is a specialized synapse that is the point of connection between motor neurons and skeletal muscle. Although developmental studies have established the importance of cell–cell communication at the NMJ for the integrity and full functionality of this synapse, the contribution of this structure as a primary driver in motor neuron disease pathogenesis remains uncertain. Here, we consider the biology of the NMJ and review emerging lines of investigation that are highlighting the importance of cell–cell interaction at the NMJ in spinal muscular atrophy (SMA), X-linked spinal and bulbar muscular atrophy (SBMA), and amyotrophic lateral sclerosis (ALS). Ongoing research may reveal NMJ targets and pathways whose therapeutic modulation will help slow the progression of motor neuron disease, offering a novel treatment paradigm for ALS, SBMA, SMA, and related disorders. The neuromuscular junction (NMJ) is a specialized synapse that is the point of connection between motor neurons and skeletal muscle. Although developmental studies have established the importance of cell–cell communication at the NMJ for the integrity and full functionality of this synapse, the contribution of this structure as a primary driver in motor neuron disease pathogenesis remains uncertain. Here, we consider the biology of the NMJ and review emerging lines of investigation that are highlighting the importance of cell–cell interaction at the NMJ in spinal muscular atrophy (SMA), X-linked spinal and bulbar muscular atrophy (SBMA), and amyotrophic lateral sclerosis (ALS). Ongoing research may reveal NMJ targets and pathways whose therapeutic modulation will help slow the progression of motor neuron disease, offering a novel treatment paradigm for ALS, SBMA, SMA, and related disorders." @default.
• W3080518452 created "2020-09-01" @default.
• W3080518452 creator A5064140449 @default.
• W3080518452 creator A5087340412 @default.
• W3080518452 date "2020-09-01" @default.
• W3080518452 modified "2023-09-25" @default.
• W3080518452 title "Harmony Lost: Cell–Cell Communication at the Neuromuscular Junction in Motor Neuron Disease" @default.
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