New insights into SMA pathogenesis: immune dysfunction and neuroinflammation
نویسندگان
چکیده
Spinal muscular atrophy (SMA) is a neuromuscular disorder characterized by motor neuron degeneration, although defects in multiple cell types and tissues have also been implicated. Three independent laboratories recently identified immune organ defects in SMA. We therefore propose a novel pathogenic mechanism contributory to SMA, resulting in higher susceptibility to infection and exacerbated disease progression caused by neuroinflammation. Overall, compromised immune function could significantly affect survival and quality of life of SMA patients. We highlight the recent findings in immune organ defects, their potential consequences on patients, our understanding of neuroinflammation in SMA, and new research hypotheses in SMA pathogenesis.
منابع مشابه
P101: Neuroinflammation and Cognitive Dysfunction as a Side Effect of Abdominal Surgery
One of the most common postoperative complications is Postoperative cognitive dysfunction (POCD), and it’s usually a geriatric’s disease. Although many studies have done, the exact molecular mechanisms of POCD is still largely unknown; nevertheless, neuroinflammation has been increasingly denoted as one of the core mechanisms for the pathogenesis of POCD. As a hypothesize, surgery-i...
متن کاملP 153: Neuroinflammation in Multiple Sclerosis
Multiple sclerosis (MS) is a complex disease which is correlated with increasing inflammatory factors, demyelination and axonal loss. In this auto-immune disease, Neuroinflammation is mediated by different types of T cells with macrophage/microglial activation and B cells involvement that interact in a collaborative manner. Focal inflammation is the main cause for the onset of relapses and coul...
متن کاملNeuroinflammation in Autism Spectrum Disorders: the Role of High Mobility Group Box 1 Protein
The pathogenesis of autism spectrum disorder (ASD) likely involves genetic and environmental factors, impacting the complex neurodevelopmental and behavioral abnormalities of the disorder. Scientific researches emerging within the past two decades suggest that immune dysfunction and inflammation have pathogenic influences through different mechanisms, all leading to both a chronic state of low ...
متن کاملP77: NADPH Oxidase Type 4 Inhibits Immune Cell Trafficking into The Central Nervous System During Neuroinflammation
Transendothelial trafficking of immune cells into the central nervous system (CNS) and disruption of the blood brain barrier (BBB) are pathophysiological hallmarks of neuroinflammatory disorders like multiple sclerosis (MS). Accumulating evidence suggest that oxidative stress plays a major role in the pathogenesis of MS, whereas a specific influence of oxidative stress on BBB dysfunction in MS ...
متن کاملP 67: The Role of Neuroinflammation in Dysfunction of Adult Hippocampal Neurogenesis
Neuroinflammation as a protective mechanism for repairing tissue damage in the central nervous system (CNS), has been classified into two types: acute and chronic. It is characterized by the activation of microglia and astrocytes and the increase levels of different chemokines and cytokines. Neuroinflammation can be harmful, and it is a common pathological feature in neurodegenerative and psych...
متن کامل