Showing papers in "Molecular and Cellular Neuroscience in 2013"

TL;DR: It is demonstrated that motor neurons derived from three sALS patients show de novo TDP-43 aggregation and that the aggregates recapitulate pathology in postmortem tissue from one of the same patients from which the iPSC were derived.

TL;DR: It is found that both MAM function and ER-mitochondrial connectivity are increased significantly in AD, which may help explain many of the biochemical and morphological features of the disease.

TL;DR: This review draws reference to biochemical, cellular and animal disease models, as well as clinical observations to elucidate the role of the innate immune system in psychiatric disorders and highlights future directions for studies, including the use of peripheral biomarkers relevant for developing new therapeutic approaches for treating psychiatric illnesses.

TL;DR: This review summarises the current knowledge regarding neuroinflammation, peripheral immune involvement, and endothelial changes in ALS.

TL;DR: The evidence shows that ASD is associated with altered neuroinflammatory processes and abnormal immune responses in adulthood, and the chronic peripheral and central alterations observed in the inflammatory response in ASD are reviewed.

TL;DR: Mechanistic studies showed that HNE-modification apparently increased the interaction of extracellular αSyn with neurons, and this suggests a specific pathological effect of H NE-αSyn on dopaminergic neurons.

TL;DR: It is suggested that ALS-F US is caused by a selective dysfunction of FUS, while FTLD-FUS may be caused by the dysfunction of the entire FET family.

TL;DR: The therapeutic role of mitochondrial bioenergetic agents such as creatine, Coenzyme-Q10, mitochondrial targeted antioxidants and peptides, the SIRT1 activator resveratrol, and the pan-PPAR agonist bezafibrate in toxin and genetic cellular and animal models of PD and HD are discussed.

TL;DR: It is concluded that the chemorepulsive axon guidance molecule Sema3A is present in PNNs of the adult rodent brain, bound to the GAGs ofThe CSPGs.

TL;DR: How defects in ISC synthesis could be a major contributor to the pathophysiology of FRDA via (i) loss of ISC-dependent enzymes, (ii) mitochondrial and cellular iron dysregulation, and (iii) enhanced iron-mediated oxidative stress is discussed.

TL;DR: This study indicates Wnt/β-catenin signaling pathway as an essential driver of glioma tumorigenesis, recognizing role of Wnt3a as an oncogene and thereby offering novel therapeutic strategies for management of these tumors.

TL;DR: Three putative models whereby PINK1 and Parkin may affect mitophagy are presented; 1) by shifting the balance between fusion and fission of the mitochondrial network, 2) by modulating mitochondrial motility and 3) by directly recruiting the autophagic machinery to damaged mitochondria.

TL;DR: A particular role of IL-17 in mechanical hyperalgesia is shown, and it is suggested that this effect is linked to an activation and upregulation of TRPV4.

TL;DR: This review intends to explore the diverse RNA-related mechanisms contributing to neurodegeneration, with a special emphasis on findings emerging from animal models.

TL;DR: A brief update on mitochondrial function and dysfunction in neurodegeneration is provided in the light of newly discovered genes associated to familial ALS and of a deeper knowledge of the mechanisms of derangement of mitochondria.

TL;DR: By manipulation of pre-mRNA splicing using antisense oligonucleotides, defective transcripts from the DMD gene and from the SMN2 gene in SMA can be modified to once again produce protein and restore function.

TL;DR: An overview of optic nerve pathology in these different clinical settings is provided, and the possible mechanisms involved in the pathogenesis of optic atrophy are reviewed, to suggest a model of general value for the field of neurodegeneration.

TL;DR: The role of one of the best-known members of this protein family, hnRNP A1, in RNA metabolism, is focused on, and recent works that note its multileveled involvement in several neurodegenerative disorders are addressed.

TL;DR: It is demonstrated for the first time that fluoxetine and citalopram decrease the release of glutamate and d-serine from LPS-activated microglia and this causes an increase in the survival of OGD-injured cortical neurons after co-culture.

TL;DR: Recent studies elucidating molecular mechanisms and biological significance of alternative pre-mRNA splicing strategies with a specific focus on their roles in nervous system development and physiology are reviewed.

TL;DR: It is found that at concentrations found in the ischemic brain, tPA interacts with synaptic but not extrasynaptic NMDARs, and Atf3 down-regulation abrogates the protective effect of tPA against excitotoxin-induced neuronal death.

TL;DR: If so, the use of benfotiamine could provide a safe intervention to reverse biological and clinical processes of AD progression, and related mechanisms may lead to reversal of plaque formation by benfutiamine in animals.

TL;DR: The effect of immune activation arising from systemic inflammatory conditions and infection, how it affects the incidence and outcomes of stroke, and the possible underlying mechanisms involved are focused on.

TL;DR: The current understanding of the mechanisms and factors involved in mammalian mitochondrial translation, and the diverse pathologies resulting when it malfunctions are discussed.

TL;DR: The practical implementation of iPSCs to study HD is examined, such as approaches to differentiate embryonic stem cells (ESCs) or iPSC into medium spiny neurons, the cell type most susceptible in HD.

TL;DR: Results indicate that IGF-1 is a growth factor that efficiently regulates different mechanisms through different downstream cascades, thereby protecting cochlear hair cells.

TL;DR: It is proposed that one mechanism by which BDNF promotes dendritic spine formation is through an interaction with Wnt signaling, and it is shown that BDNF regulates expression of Wnt1, and that Wnt2 is sufficient to promote cortical dendrite growth and dendrites formation.

TL;DR: This review will examine the normal role of neuropoietic cytokines and the potential contribution of inflammatory insults to a number of neurodevelopmental disorders, and discuss the potential for developmental inflammation to sensitise the brain to later insult.

TL;DR: The biological functions associated with neuronal ELAV proteins along evolution are reviewed and commented on and it is shown that the post-transcriptional regulatory network mediated by these RBPs in the brain is highly complex and only at an initial stage of being fully understood.

TL;DR: This review summarizes what is known about the expression of different CELF proteins in the nervous system and the evidence that they are important in neural development and function and the known targets of CELF-mediated alternative splicing regulation in the central nervous system.