Molecular classification of amyotrophic lateral sclerosis by unsupervised clustering of gene expression in motor cortex.

abstract：:Mechanisms determining characteristic age-of-onset for neurological diseases are largely unknown. Normal brain aging associates with robust and progressive transcriptome changes ("molecular aging"), but the intersection with disease pathways is mostly uncharacterized. Here, using cross-cohort microarray analysis of fo...

journal_title：Neurobiology of disease

authors： Glorioso C,Oh S,Douillard GG,Sibille E

更新日期：2011-02-01 00:00:00

abstract：:Tardive Dyskinesia (TD) is a hyperkinetic movement disorder caused by chronic treatment of psychiatric patients with dopamine (DA) receptor blocking drugs (Stacy & Jankovic 1991). Although TD is one of the most important and frequently encountered iatrogenic disorders in clinical medicine, its pathophysiology is poorl...

journal_title：Neurobiology of disease

authors： Solbrig MV,Koob GF,Fallon JH,Lipkin WI

更新日期：1994-12-01 00:00:00

abstract：:Parkinson's Disease (PD) and Multiple System Atrophy (MSA) are neurodegenerative diseases characterized neuropathologically by alpha-synuclein accumulation in brain cells. This accumulation is hypothesized to contribute to constitutive neuroinflammation, and to participate in the neurodegeneration. Cytokines, which ar...

journal_title：Neurobiology of disease

authors： Rydbirk R,Elfving B,Andersen MD,Langbøl MA,Folke J,Winge K,Pakkenberg B,Brudek T,Aznar S

更新日期：2017-10-01 00:00:00

abstract：:The mutilated-foot rat (mf rat) is an autosomal recessive mutant with characteristic digit deformities in adult animals, and this phenotype mimics many aspects of human sensory neuropathy. The genetics of mf rats was recently elucidated. To understand whether the genotype is responsible for cutaneous denervation befor...

journal_title：Neurobiology of disease

authors： Hsu SH,Lee MJ,Hsieh SC,Scaravilli F,Hsieh ST

更新日期：2004-07-01 00:00:00

abstract：:Dystonias are movement disorders whose pathomechanism is largely unknown. Dystonic dt(sz) hamsters represent a model of primary dystonias, where alterations of striatal interneuron density and sodium channel function in projection neurones were described. Here, using cortico-striatal slices, we explore whether also th...

journal_title：Neurobiology of disease

authors： Köhling R,Koch UR,Hamann M,Richter A

更新日期：2004-06-01 00:00:00

abstract：:We have examined the pattern of striatal messenger RNA expression of over 8000 genes in a rat model of levodopa (L-DOPA)-induced dyskinesia and Parkinson disease (PD). 6-Hydroxydopamine (6-OHDA)-lesioned rats were treated with L-DOPA or physiological saline for 22 days and repeatedly tested for antiakinetic response t...

journal_title：Neurobiology of disease

authors： Konradi C,Westin JE,Carta M,Eaton ME,Kuter K,Dekundy A,Lundblad M,Cenci MA

更新日期：2004-11-01 00:00:00

abstract：:The critical anabolic and trophic role of signaling by insulin-like growth factors (IGF) I and II via the type-I IGF receptor (IGF-IR) is reviewed throughout the life of skeletal myocytes. The proliferative effects of IGF-IR stimulation, both during embryogenesis and during satellite cell proliferation following dener...

journal_title：Neurobiology of disease

authors： Singleton JR,Feldman EL

更新日期：2001-08-01 00:00:00

abstract：:There is evidence that cognitive decline in Alzheimer's disease (AD) results from deficiencies in synaptic communication (e.g., loss of mushroom-shaped 'memory spines') and neurodegenerative processes. This might be treated with sigma-1 receptor (S1R) agonists, which are broadly neuroprotective and modulate synaptic p...

journal_title：Neurobiology of disease

authors： Ryskamp D,Wu L,Wu J,Kim D,Rammes G,Geva M,Hayden M,Bezprozvanny I

更新日期：2019-04-01 00:00:00

abstract：:Converging lines of evidence suggest that neuroinflammatory processes may account for the progressive death of dopaminergic neurons in Parkinson's disease (PD). Therefore, anti-inflammatory strategies have attracted much interest for their potential to prevent further deterioration of PD. Our previous study showed tha...

journal_title：Neurobiology of disease

authors： Zhou HF,Liu XY,Niu DB,Li FQ,He QH,Wang XM

更新日期：2005-04-01 00:00:00

abstract：:In amyotrophic lateral sclerosis (ALS), the exogenous temporal triggers that result in initial motor neuron death are not understood. Overactivation and consequent accelerated loss of vulnerable motor neurons is one theory of disease initiation. The vulnerability of motor neurons in response to chronic peripheral nerv...

journal_title：Neurobiology of disease

authors： Lepore AC,Tolmie C,O'Donnell J,Wright MC,Dejea C,Rauck B,Hoke A,Ignagni AR,Onders RP,Maragakis NJ

更新日期：2010-09-01 00:00:00

abstract：:Mental illness is the leading cause of disability worldwide. We are only just beginning to reveal and comprehend the complex interaction that exists between the genetic makeup of an organism and the potential modifying effect of the environment in which it lives, and how this translates into mediating susceptibility t...

journal_title：Neurobiology of disease

authors： Renoir T,Pang TY,Hannan AJ

更新日期：2013-09-01 00:00:00

abstract：:Necroptosis is a regulated form of necrosis that is mediated by a variety of proteins including tumor necrosis factor-α (TNF-α) and receptor-interacting proteins (RIPs). TNF-α, a critical inflammatory molecule, is one of the initiating signals in the necroptosis pathway, and RIP3 acts as a switch that commits the cell...

journal_title：Neurobiology of disease

authors： Yuan S,Yu Z,Zhang Z,Zhang J,Zhang P,Li X,Li H,Shen H,Chen G

更新日期：2019-09-01 00:00:00

abstract：:Possible roles of the complement (C) system in the normal and injured brain were explored with inbred mice that carried a frameshift mutation in the C5 gene. A congenic pair was used: the C5-sufficient (C5+) B10.D2/nSnJ strain with the functional allele (Hc1) from the C57BL/10J donor strain was compared with the C5-de...

journal_title：Neurobiology of disease

authors： Pasinetti GM,Tocco G,Sakhi S,Musleh WD,DeSimoni MG,Mascarucci P,Schreiber S,Baudry M,Finch CE

更新日期：1996-01-01 00:00:00

abstract：:Polyglutamine neurodegenerative disorders are characterized by the expansion of a glutamine tract within the mutant disease-causing protein. Expression of the mutant protein induces a progressive loss of neuronal function and the subsequent neurodegeneration of a set of neurons characteristic to each disease. Spinocer...

journal_title：Neurobiology of disease

authors： Orr HT

更新日期：2000-06-01 00:00:00

abstract：:There is no information concerning signal transduction mechanisms downstream of the opioid/nociceptin receptors in the human epileptic brain. The aim of this work was to evaluate the level of G-proteins activation mediated by DAMGO (a mu receptor selective peptide) and nociceptin, and the binding to mu and nociceptin ...

journal_title：Neurobiology of disease

authors： Rocha L,Orozco-Suarez S,Alonso-Vanegas M,Villeda-Hernandez J,Gaona A,Páldy E,Benyhe S,Borsodi A

更新日期：2009-09-01 00:00:00

abstract：BACKGROUND/INTRODUCTION:The neuroinflammatory response plays a major role in the secondary injury cascade after traumatic spinal cord injury (SCI). To date, systemic anti-inflammatory medications such as methylprednisolone sodium succinate (MPSS) have shown promise in SCI. However, systemic immunosuppression can have d...

journal_title：Neurobiology of disease

authors： Chio JCT,Wang J,Surendran V,Li L,Zavvarian MM,Pieczonka K,Fehlings MG

更新日期：2021-01-01 00:00:00

abstract：:The motor cortex and subthalamic nucleus (STN) of patients with Parkinson's disease (PD) exhibit abnormally high levels of electrophysiological oscillations in the ~12-35 Hz beta-frequency range. Recent studies have shown that beta is partly carried forward to regulate future motor states in the healthy condition, sug...

journal_title：Neurobiology of disease

authors： Polar CA,Gupta R,Lehmkuhle MJ,Dorval AD

更新日期：2018-09-01 00:00:00

abstract：:Friedreich ataxia (FRDA) is an autosomal recessive degenerative disease caused either by an intronic GAA triplet repeat expansion that suppresses the expression of the frataxin gene on chromosome 9q13, or, rarely, by point mutations in the frataxin gene. We investigated the expression of the mouse frataxin homologue d...

journal_title：Neurobiology of disease

authors： Jiralerspong S,Liu Y,Montermini L,Stifani S,Pandolfo M

更新日期：1997-01-01 00:00:00

abstract：:Bradykinesia is the most important feature contributing to motor difficulties in Parkinson's disease (PD). However, the pathophysiology underlying bradykinesia is not fully understood. One important aspect is that PD patients have difficulty in performing learned motor skills automatically, but this problem has been g...

journal_title：Neurobiology of disease

authors： Wu T,Hallett M,Chan P

更新日期：2015-10-01 00:00:00

abstract：:We investigated the effects of engrafted human adult olfactory neuroepithelial neurosphere forming cells (NSFCs) on regeneration and reinnervation of rubrospinal tract (RST) axons and locomotor recovery following partial cervical hemisection that completely ablated the RST. Weekly behavioral testing showed greater fun...

journal_title：Neurobiology of disease

authors： Xiao M,Klueber KM,Guo Z,Lu C,Wang H,Roisen FJ

更新日期：2007-05-01 00:00:00

abstract：:The dysregulation of glycogen synthase kinase-3 (GSK3) has been implicated in Alzheimer disease (AD) pathogenesis and in Abeta-induced neurotoxicity, leading us to investigate it as a therapeutic target in an intracerebroventricular Abeta infusion model. Infusion of a specific GSK3 inhibitor SB216763 (SB) reduced a do...

journal_title：Neurobiology of disease

authors： Hu S,Begum AN,Jones MR,Oh MS,Beech WK,Beech BH,Yang F,Chen P,Ubeda OJ,Kim PC,Davies P,Ma Q,Cole GM,Frautschy SA

更新日期：2009-02-01 00:00:00

abstract：:Voltage-gated sodium channels (VGSCs) are essential for the generation and propagation of action potentials in electrically excitable cells. Dominant mutations in SCN1A, which encodes the Nav1.1 VGSC α-subunit, underlie several forms of epilepsy, including Dravet syndrome (DS) and genetic epilepsy with febrile seizure...

journal_title：Neurobiology of disease

authors： Dutton SB,Makinson CD,Papale LA,Shankar A,Balakrishnan B,Nakazawa K,Escayg A

更新日期：2013-01-01 00:00:00

abstract：:Cellular mechanisms play a role in conversion of the normal prion protein PrP(C) to the disease-associated protein PrP(Sc). The cells provide not only PrP(C), but also still largely undefined factors required for efficient prion replication. Previously, we have observed that interference with ERK and p38-JNK MAP kinas...

journal_title：Neurobiology of disease

authors： Allard EK,Grujic M,Fisone G,Kristensson K

更新日期：2013-10-01 00:00:00

abstract：:In this review, we highlight critical unresolved questions in the etiology and mechanisms causing preterm brain injury. Involvement of neurons, glia, endogenous factors and exogenous exposures is considered. The structural and functional correlates of interrupted development and injury in the premature brain are under...

journal_title：Neurobiology of disease

authors： Penn AA,Gressens P,Fleiss B,Back SA,Gallo V

更新日期：2016-08-01 00:00:00

abstract：:It has been proposed that Alzheimer disease (AD) is associated with a "disconnection syndrome" due to the gradual loss of morphological and functional integrity of cortico-cortical pathways. This hypothesis derives from indirect neuropathological observations, but definitive evidence that AD primarily targets cortico-...

journal_title：Neurobiology of disease

authors： Delatour B,Blanchard V,Pradier L,Duyckaerts C

更新日期：2004-06-01 00:00:00

abstract：:Recent studies indicate that the Parkinson's disease-linked leucine-rich repeat kinase 2 (LRRK2) modulates cytoskeletal functions by regulating actin and tubulin dynamics, thereby affecting neurite outgrowth. By interactome analysis we demonstrate that the binding of LRRK2 to tubulins is significantly enhanced by phar...

journal_title：Neurobiology of disease

authors： Caesar M,Zach S,Carlson CB,Brockmann K,Gasser T,Gillardon F

更新日期：2013-06-01 00:00:00

abstract：:Charcot-Marie-Tooth disease (CMT) is a genetic disorder that can be caused by aberrations in >80 genes. CMT has heterogeneous modes of inheritance, including autosomal dominant, autosomal recessive, X-linked dominant, and X-linked recessive. Over 95% of cases are dominantly inherited. In this study, we investigated wh...

journal_title：Neurobiology of disease

authors： Lee JS,Chang EH,Koo OJ,Jwa DH,Mo WM,Kwak G,Moon HW,Park HT,Hong YB,Choi BO

更新日期：2017-04-01 00:00:00

abstract：:Studies have showed that prenatal cocaine exposure (PCOC) can impair cognitive function and social behavior of the offspring; however, the mechanism underlying such effect is poorly understood. Insulin-like growth factor II (Igf-II), an imprinted gene, has a critical role in memory consolidation and enhancement. We hy...

journal_title：Neurobiology of disease

authors： Zhao Q,Hou J,Chen B,Shao X,Zhu R,Bu Q,Gu H,Li Y,Zhang B,Du C,Fu D,Kong J,Luo L,Long H,Li H,Deng Y,Zhao Y,Cen X

更新日期：2015-10-01 00:00:00

abstract：:Neurodegenerative diseases impose a burden on society, yet for the most part, the mechanisms underlying neuronal dysfunction and death in these disorders remain unclear despite the identification of relevant disease genes. Given the molecular conservation in neuronal signaling pathways across vertebrate and invertebra...

journal_title：Neurobiology of disease

authors： Dimitriadi M,Hart AC

更新日期：2010-10-01 00:00:00

abstract：:Intermittent hypoxia (IH) during sleep induces temporally defined increases in apoptosis within vulnerable brain regions such as the hippocampal CA1 region in rats. Protein kinase B (AKT) has emerged as major signal transduction protein underlying inhibition of apoptosis and consequent increases in cell survival. Spra...

journal_title：Neurobiology of disease

authors： Goldbart A,Cheng ZJ,Brittian KR,Gozal D

更新日期：2003-12-01 00:00:00