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Vir B Singh et al.
Scientific reports, 6, 26876-26876 (2016-06-01)
Human Immunodeficiency Virus type-1 (HIV)-associated neurocognitive disorder is characterized by recruitment of activated/infected leukocytes into the CNS via disrupted Blood Brain Barrier (BBB) that contributes to persistent neuro-inflammation. In this report, humanized NOD/scid-IL2Rγc(null) mice were used to establish that impaired
Pathogenic POGZ mutation causes impaired cortical development and reversible autism-like phenotypes.
Kensuke Matsumura et al.
Nature communications, 11(1), 859-859 (2020-02-28)
Pogo transposable element derived with ZNF domain (POGZ) has been identified as one of the most recurrently de novo mutated genes in patients with neurodevelopmental disorders (NDDs), including autism spectrum disorder (ASD), intellectual disability and White-Sutton syndrome; however, the neurobiological
Xin-Ming Ma et al.
Molecular biology of the cell, 25(9), 1458-1471 (2014-03-07)
Like several Rho GDP/GTP exchange factors (GEFs), Kalirin7 (Kal7) contains an N-terminal Sec14 domain and multiple spectrin repeats. A natural splice variant of Kalrn lacking the Sec14 domain and four spectrin repeats is unable to increase spine formation; our goal
Barış Genç et al.
Scientific reports, 7, 41765-41765 (2017-02-07)
Apical dendrites of Betz cells are important sites for the integration of cortical input, however their health has not been fully assessed in ALS patients. We investigated the primary motor cortices isolated from post-mortem normal control subjects, patients with familial
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Gilbert Gallardo et al.
Nature neuroscience, 17(12), 1710-1719 (2014-10-27)
Perturbations of astrocytes trigger neurodegeneration in several diseases, but the glial cell-intrinsic mechanisms that induce neurodegeneration remain poorly understood. We found that a protein complex of α2-Na/K ATPase and α-adducin was enriched in astrocytes expressing mutant superoxide dismutase 1 (SOD1)
Knut H Lauritzen et al.
Neurobiology of aging, 48, 34-47 (2016-09-18)
Mitochondrial genome maintenance plays a central role in preserving brain health. We previously demonstrated accumulation of mitochondrial DNA damage and severe neurodegeneration in transgenic mice inducibly expressing a mutated mitochondrial DNA repair enzyme (mutUNG1) selectively in forebrain neurons. Here, we
Felicia L Smith et al.
The Journal of comparative neurology, 524(11), 2182-2207 (2015-12-15)
The spiral ganglion is a compelling model system to examine how morphological form contributes to sensory function. While the ganglion is composed mainly of a single class of type I neurons that make simple one-to-one connections with inner hair cell
Xiling Yin et al.
Bio-protocol, 10(17), e3746-e3746 (2021-03-05)
Human neuron transplantation offers novel opportunities for modeling human neurologic diseases and potentially replacement therapies. However, the complex structure of the human cerebral cortex, which is organized in six layers with tightly interconnected excitatory and inhibitory neuronal networks, presents significant
Dian Yang et al.
eLife, 8 (2019-12-14)
Metastasis is the main cause of death in cancer patients but remains a poorly understood process. Small cell lung cancer (SCLC) is one of the most lethal and most metastatic cancer types. SCLC cells normally express neuroendocrine and neuronal gene
Hei Ming Lai et al.
PloS one, 11(6), e0158628-e0158628 (2016-07-01)
Three-dimensional visualization of intact tissues is now being achieved by turning tissues transparent. CLARITY is a unique tissue clearing technique, which features the use of detergents to remove lipids from fixed tissues to achieve optical transparency. To preserve tissue integrity
Angela L Rachubinski et al.
PloS one, 7(4), e36082-e36082 (2012-05-05)
As much of the aberrant neural development in Down syndrome (DS) occurs postnatally, an early opportunity exists to intervene and influence life-long cognitive development. Recent success using neural progenitor cells (NPC) in models of adult neurodegeneration indicate such therapy may
Characterization of amyloid-? granules in the hippocampus of SAMP8 mice.
Manich, Gemma, et al.
Journal of Alzheimer'S Disease, 25, 535-546 (2011)
Gerardo Rosciszewski et al.
Frontiers in cellular neuroscience, 13, 380-380 (2019-09-12)
Temporal Lobe Epilepsy (TLE) is the most common form of human epilepsy and available treatments with antiepileptic drugs are not disease-modifying therapies. The neuroinflammation, neuronal death and exacerbated plasticity that occur during the silent period, following the initial precipitating event
Dopaminergic neuronal conversion from adult rat skeletal muscle-derived stem cells in vitro.
Jian Yang,Xuan Wang,Yue Wang,Zi-Xuan Guo,Ding-Zhen Luo,Jun Jia,Xiao-Min Wang
Neurochemical Research null
Endogenous cerebellar neurogenesis in adult mice with progressive ataxia.
Kumar, M; Csaba, Z; Peineau, S; Srivastava, R; Rasika, S; Mani, S; Gressens, P; El Ghouzzi, V
Annals of Clinical and Translational Neurology null
Hideyuki Nakashima et al.
The Journal of neuroscience : the official journal of the Society for Neuroscience, 38(20), 4791-4810 (2018-04-27)
Functional neuronal connectivity requires proper neuronal morphogenesis and its dysregulation causes neurodevelopmental diseases. Transforming growth factor-β (TGF-β) family cytokines play pivotal roles in development, but little is known about their contribution to morphological development of neurons. Here we show that
Gwen B Effgen et al.
Journal of neurotrauma, 33(20), 1901-1912 (2016-10-21)
Up to 80% of injuries sustained by U.S. soldiers in Operation Enduring Freedom and Operation Iraqi Freedom were the result of blast exposure from improvised explosive devices. Some soldiers experience multiple blasts while on duty, and it has been suggested
Jonathan D Driscoll et al.
Journal of neurophysiology, 105(6), 3106-3113 (2011-04-08)
We present a high-speed photon counter for use with two-photon microscopy. Counting pulses of photocurrent, as opposed to analog integration, maximizes the signal-to-noise ratio so long as the uncertainty in the count does not exceed the gain-noise of the photodetector.
Differentiated human midbrain-derived neural progenitor cells express excitatory strychnine-sensitive glycine receptors containing ?2? subunits.
Wegner, F; Kraft, R; Busse, K; Hartig, W; Ahrens, J; Leffler, A; Dengler, R; Schwarz, J
Testing null
Carmen Falcone et al.
Pediatric research, 87(5), 868-871 (2019-11-30)
The presence and status of progenitor/stem cells in excencephalic brain have not been previously examined. Brain sections of excencephalic 17-week fetus were stained for specific stem and mature cell markers. The ventricles were open, the developing cerebral cortex was thin
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Christian Wüthrich et al.
Journal of neuropathology and experimental neurology, 72(11), 1043-1051 (2013-10-17)
Natalizumab, a monoclonal antibody directed against α4 integrins, has, to date, been associated with 399 cases of progressive multifocal leukoencephalopathy (PML) worldwide in patients receiving treatment for multiple sclerosis (MS). Because of the limited number of histologic studies, the possible
Matthew T Simpson et al.
Molecular and cellular neurosciences, 68, 272-283 (2015-08-27)
Neurons in the embryonic and peripheral nervous system respond to injury by activating transcriptional programs supportive of axon growth, ultimately resulting in functional recovery. In contrast, neurons in the adult central nervous system (CNS) possess a limited capacity to regenerate
Julbert Caneus et al.
Alzheimer's & dementia (New York, N. Y.), 6(1), e12029-e12029 (2020-06-04)
The quest to identify an effective therapeutic strategy for neurodegenerative diseases, such as mild congitive impairment (MCI) and Alzheimer's disease (AD), suffers from the lack of good human-based models. Animals represent the most common models used in basic research and
Gen Shiihashi et al.
EBioMedicine, 24, 102-115 (2017-09-21)
Cytoplasmic aggregation of fused in sarcoma (FUS) is detected in brain regions affected by amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), which compose the disease spectrum, FUS proteinopathy. To understand the pathomechanism of ALS-FTD-associated FUS, we examined the behavior
Role of mismatch repair enzymes in GAA?TTC triplet-repeat expansion in Friedreich ataxia induced pluripotent stem cells.
Du, J; Campau, E; Soragni, E; Ku, S; Puckett, JW; Dervan, PB; Gottesfeld, JM
The Journal of Biological Chemistry null
Ying Sun et al.
PloS one, 10(3), e0118771-e0118771 (2015-03-31)
Gaucher disease (GD) is caused by insufficient activity of acid β-glucosidase (GCase) resulting from mutations in GBA1. To understand the pathogenesis of the neuronopathic GD, induced pluripotent stem cells (iPSCs) were generated from fibroblasts isolated from three GD type 2
Kimberly P Keil et al.
Toxicological sciences : an official journal of the Society of Toxicology, 168(1), 95-109 (2018-11-06)
Early life exposures to environmental contaminants are implicated in the pathogenesis of many neurodevelopmental disorders (NDDs). These disorders often display sex biases, but whether environmental neurotoxicants act in a sex-dependent manner to modify neurodevelopment is largely unknown. Since altered dendritic
Edit Hathy et al.
Stem cell research & therapy, 11(1), 504-504 (2020-11-29)
De novo mutations (DNMs) have been implicated in the etiology of schizophrenia (SZ), a chronic debilitating psychiatric disorder characterized by hallucinations, delusions, cognitive dysfunction, and decreased community functioning. Several DNMs have been identified by examining SZ cases and their unaffected
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