当前位置： SCI文献检索 > NEUROBIOLOGY OF DISEASE期刊下所有文献 > Spinocerebellar ataxia: miRNAs expose biological pathways underlying pervasive Purkinje cell degeneration.

Spinocerebellar ataxia: miRNAs expose biological pathways underlying pervasive Purkinje cell degeneration.

Abstract：

:Recent work has demonstrated the importance of miRNAs in the pathogenesis of various brain disorders including the neurodegenerative disorder spinocerebellar ataxia (SCA). This review focuses on the role of miRNAs in the shared pathogenesis of the different SCA types. We examine the novel findings of a recent cell-type-specific RNA-sequencing study in mouse brain and discuss how the identification of Purkinje-cell-enriched miRNAs highlights biological pathways that expose the mechanisms behind pervasive Purkinje cell degeneration in SCA. These key pathways are likely to contain targets for therapeutic development and represent potential candidate genes for genetically unsolved SCAs.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

van der Stijl R,Withoff S,Verbeek DS

doi

10.1016/j.nbd.2017.08.003

subject

Has Abstract

pub_date

2017-12-01 00:00:00

pages

148-158

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(17)30188-2

journal_volume

108

pub_type

杂志文章,评审

相关文献

NEUROBIOLOGY OF DISEASE文献大全
  • Identification of a unique apolipoprotein E allele in Microcebus murinus; ApoE brain distribution and co-localization with beta-amyloid and tau proteins.

    abstract：:This report is devoted to the characterization of the apolipoprotein E (ApoE) in Microcebus murinus. Only one allele homologous to the human ApoE4 allele was evidenced. The distribution of the corresponding ApoE protein in the brain was found in association with the pathological proteins characteristic of Alzheimer's ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1006/nbdi.1995.0018

    authors： Calenda A,Jallageas V,Silhol S,Bellis M,Bons N

    更新日期：1995-06-01 00:00:00

  • The SARS-CoV-2 spike protein alters barrier function in 2D static and 3D microfluidic in-vitro models of the human blood-brain barrier.

    abstract：:As researchers across the globe have focused their attention on understanding SARS-CoV-2, the picture that is emerging is that of a virus that has serious effects on the vasculature in multiple organ systems including the cerebral vasculature. Observed effects on the central nervous system include neurological symptom...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2020.105131

    authors： Buzhdygan TP,DeOre BJ,Baldwin-Leclair A,Bullock TA,McGary HM,Khan JA,Razmpour R,Hale JF,Galie PA,Potula R,Andrews AM,Ramirez SH

    更新日期：2020-12-01 00:00:00

  • Amyloid-beta peptide is toxic to neurons in vivo via indirect mechanisms.

    abstract：:We have studied the neurotoxicity of amyloid-beta (Abeta) after a single unilateral intravitreal injection. Within the retina apoptotic cells were seen throughout the photoreceptor layer and the inner nuclear layer but not in the ganglion cell layer at 48 h after injection of Abeta(1-42) compared to vehicle control an...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1006/nbdi.2002.0485

    authors： Walsh DT,Montero RM,Bresciani LG,Jen AY,Leclercq PD,Saunders D,EL-Amir AN,Gbadamoshi L,Gentleman SM,Jen LS

    更新日期：2002-06-01 00:00:00

  • Proliferative response of distinct hippocampal progenitor cell populations after cortical infarcts in the adult brain.

    abstract：:Precursor cells in the adult dentate gyrus are a heterogeneous population. Astrocytic cell types with radial glia-like morphology and low proliferative activity have been distinguished from highly dividing subtypes expressing early neuronal properties. Recent evidence indicates that physiological stimuli predominantly...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2005.07.013

    authors： Kunze A,Grass S,Witte OW,Yamaguchi M,Kempermann G,Redecker C

    更新日期：2006-02-01 00:00:00

  • Neuropeptide Y (NPY) as a therapeutic target for neurodegenerative diseases.

    abstract：:Neuropeptide Y (NPY) and NPY receptors are widely expressed in the mammalian central nervous system. Studies in both humans and rodent models revealed that brain NPY levels are altered in some neurodegenerative disorders, such as Alzheimer's disease, Parkinson's disease, Huntington's disease and Machado-Joseph disease...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章,评审

    doi：10.1016/j.nbd.2016.07.022

    authors： Duarte-Neves J,Pereira de Almeida L,Cavadas C

    更新日期：2016-11-01 00:00:00

  • White matter connectivity reflects clinical and cognitive status in Huntington's disease.

    abstract：OBJECTIVE:To investigate structural connectivity and the relationship between axonal microstructure and clinical, cognitive, and motor functions in premanifest (pre-HD) and symptomatic (symp-HD) Huntington's disease. METHOD:Diffusion tensor imaging (DTI) data were acquired from 35 pre-HD, 36 symp-HD, and 35 controls. ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2014.01.013

    authors： Poudel GR,Stout JC,Domínguez D JF,Salmon L,Churchyard A,Chua P,Georgiou-Karistianis N,Egan GF

    更新日期：2014-05-01 00:00:00

  • Inverse association between CSF Aβ 42 levels and years of education in mild form of Alzheimer's disease: the cognitive reserve theory.

    abstract：:In Alzheimer's disease (AD), the cognitive reserve theory predicts that at any level of assessed clinical severity, the underlying brain pathology is more advanced in patients with more cognitive reserve. Recent evidences suggest that cerebrospinal fluid (CSF) biomarkers may reflect the brain pathology in AD. We inves...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2010.07.007

    authors： Dumurgier J,Paquet C,Benisty S,Kiffel C,Lidy C,Mouton-Liger F,Chabriat H,Laplanche JL,Hugon J

    更新日期：2010-11-01 00:00:00

  • Modulation of serotonin dynamics in the dorsal raphe nucleus via high frequency medial prefrontal cortex stimulation.

    abstract：:The subcallosal cingulate (SCC) region, or its rodent homologue the medial prefrontal cortex (mPFC), and midbrain dorsal raphe (DR) are crucial nodes of the widespread network implicated in emotional regulation. Stimulation of the SCC is being explored as a potential treatment for depression. Because modulation of the...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2016.06.009

    authors： Srejic LR,Wood KM,Zeqja A,Hashemi P,Hutchison WD

    更新日期：2016-10-01 00:00:00

  • Prostaglandin J2 reduces catechol-O-methyltransferase activity and enhances dopamine toxicity in neuronal cells.

    abstract：:There is clear evidence that an inflammatory reaction is mounted within the CNS following trauma, stroke, infection and seizures, thus augmenting brain damage. Furthermore, chronic inflammation of the CNS is implicated in many neurodegenerative disorders. However, the effects of products of inflammation on neuronal ce...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2005.11.006

    authors： Ogburn KD,Bottiglieri T,Wang Z,Figueiredo-Pereira ME

    更新日期：2006-05-01 00:00:00

  • Deletion of exons 9 and 10 of the Presenilin 1 gene in a patient with Early-onset Alzheimer Disease generates longer amyloid seeds.

    abstract：:Presenilin 1 (PSEN1) mutations are the main cause of autosomal dominant Early-onset Alzheimer Disease (EOAD). Among them, deletions of exon 9 have been reported to be associated with a phenotype of spastic paraparesis. Using exome data from a large sample of 522 EOAD cases and 584 controls to search for genomic copy-n...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2017.04.020

    authors： Le Guennec K,Veugelen S,Quenez O,Szaruga M,Rousseau S,Nicolas G,Wallon D,Fluchere F,Frébourg T,De Strooper B,Campion D,Chávez-Gutiérrez L,Rovelet-Lecrux A

    更新日期：2017-08-01 00:00:00

  • Implications of gut microbiota dysbiosis and metabolic changes in prion disease.

    abstract：:Evidence of the gut microbiota influencing neurodegenerative diseases has been reported for several neural diseases. However, there is little insight regarding the relationship between the gut microbiota and prion disease. Here, using fecal samples of 12 prion-infected mice and 25 healthy controls, we analyzed the str...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2019.104704

    authors： Yang D,Zhao D,Shah SZA,Wu W,Lai M,Zhang X,Li J,Guan Z,Zhao H,Li W,Gao H,Zhou X,Yang L

    更新日期：2020-02-01 00:00:00

  • Psychosine induces the dephosphorylation of neurofilaments by deregulation of PP1 and PP2A phosphatases.

    abstract：:Patients with Krabbe disease, a genetic demyelinating syndrome caused by deficiency of galactosyl-ceramidase and the resulting accumulation of galactosyl-sphingolipids, develop signs of a dying-back axonopathy compounded by a deficiency of large-caliber axons. Here, we show that axonal caliber in Twitcher mice, an ani...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2012.01.013

    authors： Cantuti-Castelvetri L,Zhu H,Givogri MI,Chidavaenzi RL,Lopez-Rosas A,Bongarzone ER

    更新日期：2012-05-01 00:00:00

  • Molecular insights and therapeutic targets for blood-brain barrier disruption in ischemic stroke: critical role of matrix metalloproteinases and tissue-type plasminogen activator.

    abstract：:Blood-brain barrier (BBB) disruption, mediated through matrix metalloproteinases (MMPs) and other mechanisms, is a critical event during ischemic stroke. Tissue plasminogen activator (tPA) is the only FDA-approved thrombolytic therapy for acute ischemic stroke, but the efficacy and safety of its therapeutic applicatio...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章,评审

    doi：10.1016/j.nbd.2010.03.008

    authors： Jin R,Yang G,Li G

    更新日期：2010-06-01 00:00:00

  • Alzheimer's brains show inter-related changes in RNA and lipid metabolism.

    abstract：:Alzheimer's disease (AD) involves changes in both lipid and RNA metabolism, but it remained unknown if these differences associate with AD's cognition and/or post-mortem neuropathology indices. Here, we report RNA-sequencing evidence of inter-related associations between lipid processing, cognition level, and AD neuro...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2017.06.008

    authors： Barbash S,Garfinkel BP,Maoz R,Simchovitz A,Nadorp B,Guffanti A,Bennett ER,Nadeau C,Türk A,Paul L,Reda T,Li Y,Buchman AS,Greenberg DS,Seitz A,Bennett DA,Giavalisco P,Soreq H

    更新日期：2017-10-01 00:00:00

  • Preventing polyglutamine-induced activation of c-Jun delays neuronal dysfunction in a mouse model of SCA7 retinopathy.

    abstract：:We have approached the role of cellular stress in neurodegenerative diseases caused by polyglutamine expansion (polyQ) in the context of Spinocerebellar ataxia type 7 (SCA7) that includes retinal degeneration. Using the R7E mouse, in which polyQ-ataxin-7 is specifically over-expressed in rod photoreceptors, we previou...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2006.11.002

    authors： Merienne K,Friedman J,Akimoto M,Abou-Sleymane G,Weber C,Swaroop A,Trottier Y

    更新日期：2007-03-01 00:00:00

  • Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA.

    abstract：:Familial amyotrophic lateral sclerosis (FALS) has been modeled in transgenic mice by introducing mutated versions of human genomic DNA encompassing the entire gene for Cu,Zn superoxide dismutase (SOD1). In this setting, the transgene is expressed throughout the body and results in mice that faithfully recapitulate man...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2005.06.005

    authors： Wang J,Xu G,Slunt HH,Gonzales V,Coonfield M,Fromholt D,Copeland NG,Jenkins NA,Borchelt DR

    更新日期：2005-12-01 00:00:00

  • Transient receptor potential vanilloid 1 channels modulate the synaptic effects of TNF-α and of IL-1β in experimental autoimmune encephalomyelitis.

    abstract：:Transient receptor potential vanilloid 1 (TRPV1) channels are involved in several inflammatory diseases. However, their action is still controversial, and both pro-inflammatory and anti-inflammatory roles have been described. We used a strain of TRPV1-KO mice to characterize the role of these channels in experimental ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2011.05.018

    authors： Musumeci G,Grasselli G,Rossi S,De Chiara V,Musella A,Motta C,Studer V,Bernardi G,Haji N,Sepman H,Fresegna D,Maccarrone M,Mandolesi G,Centonze D

    更新日期：2011-09-01 00:00:00

  • Neuroprotective activity of ursodeoxycholic acid in CHMP2BIntron5 models of frontotemporal dementia.

    abstract：:Frontotemporal dementia (FTD) is one of the most prevalent forms of early-onset dementia. It represents part of the FTD-Amyotrophic Lateral Sclerosis (ALS) spectrum, a continuum of genetically and pathologically overlapping disorders. FTD-causing mutations in CHMP2B, a gene encoding a core component of the heteromeric...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2020.105047

    authors： West RJH,Ugbode C,Fort-Aznar L,Sweeney ST

    更新日期：2020-10-01 00:00:00

  • Lack of CCR5 modifies glial phenotypes and population of the nigral dopaminergic neurons, but not MPTP-induced dopaminergic neurodegeneration.

    abstract：:Constitutive expression of C-C chemokine receptor (CCR) 5 has been detected in astrocytes, microglia and neurons, but its physiological roles in the central nervous system are obscure. The bidirectional interactions between neuron and glial cells through CCR5 and its ligands were thought to be crucial for maintaining ...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2012.08.001

    authors： Choi DY,Lee MK,Hong JT

    更新日期：2013-01-01 00:00:00

  • Huntingtin with an expanded polyglutamine repeat affects the Jab1-p27(Kip1) pathway.

    abstract：:Expansion of polyglutamine repeats is the cause of at least nine inherited human neurodegenerative disorders, including Huntington's disease (HD). It is widely accepted that deregulation of the transcriptional coactivator CBP by expanded huntingtin (htt) plays an important role in HD molecular pathogenesis. In this st...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2012.03.008

    authors： Cong SY,Pepers BA,Zhou TT,Kerkdijk H,Roos RA,van Ommen GJ,Dorsman JC

    更新日期：2012-06-01 00:00:00

  • The brain and behavioral correlates of motor-related analgesia (MRA).

    abstract：:The human motor system has the capacity to act as an internal form of analgesia. Since the discovery of the potential influence of motor systems on analgesia in rodent models, clinical applications of targeting the motor system for analgesia have been implemented. However, a neurobiological basis for motor activation'...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章,评审

    doi：10.1016/j.nbd.2020.105158

    authors： Holmes SA,Kim A,Borsook D

    更新日期：2021-01-01 00:00:00

  • Reducing canonical Wingless/Wnt signaling pathway confers protection against mutant Huntingtin toxicity in Drosophila.

    abstract：:Huntington's disease (HD) is a genetic neurodegenerative disease characterized by movement disorders, cognitive decline and neuropsychiatric symptoms. HD is caused by expanded CAG tract within the coding region of Huntingtin protein. Despite major insights into the molecular mechanisms leading to HD, no effective cure...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2012.04.007

    authors： Dupont P,Besson MT,Devaux J,Liévens JC

    更新日期：2012-08-01 00:00:00

  • Role of the endogenous cannabinoid system in the regulation of motor activity.

    abstract：:One of the prominent pharmacological features of drugs acting at the brain cannabinoid receptor (CB1) is the induction of alterations in motor behavior. Catalepsy, immobility, ataxia, or the impairment of complex behavioral acts are observed after acute administration of either natural and synthetic cannabinoid recept...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章,评审

    doi：10.1006/nbdi.1998.0217

    authors： Rodríguez de Fonseca F,Del Arco I,Martín-Calderón JL,Gorriti MA,Navarro M

    更新日期：1998-12-01 00:00:00

  • Over-expression of tau results in defective synaptic transmission in Drosophila neuromuscular junctions.

    abstract：:We have shown that over-expression of human tau (0N3R) in Drosophila larval motor neurons causes significant morphological and functional disruption to the neuromuscular junctions (NMJs). Tau-expressing NMJs are reduced in size with irregular and abnormal bouton structure. Immunocytochemical analysis shows that the ab...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2005.05.029

    authors： Chee FC,Mudher A,Cuttle MF,Newman TA,MacKay D,Lovestone S,Shepherd D

    更新日期：2005-12-01 00:00:00

  • Transcriptome analysis in a rat model of L-DOPA-induced dyskinesia.

    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

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2004.07.005

    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

  • Adolescent cannabis exposure interacts with mutant DISC1 to produce impaired adult emotional memory.

    abstract：:Cannabis is an increasingly popular and controversial drug used worldwide. Cannabis use often begins during adolescence, a highly susceptible period for environmental stimuli to alter functional and structural organization of the developing brain. Given that adolescence is a critical time for the emergence of mental i...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2015.06.006

    authors： Ballinger MD,Saito A,Abazyan B,Taniguchi Y,Huang CH,Ito K,Zhu X,Segal H,Jaaro-Peled H,Sawa A,Mackie K,Pletnikov MV,Kamiya A

    更新日期：2015-10-01 00:00:00

  • TOC1: a valuable tool in assessing disease progression in the rTg4510 mouse model of tauopathy.

    abstract：:All tauopathies result in various forms of cognitive decline and neuronal loss. Although in some diseases, tau mutations appear to cause neurodegeneration, the toxic "form" of tau remains elusive. Tau is the major protein found within neurofibrillary tangles (NFTs) and therefore it seemed rational to assume that aggre...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2014.03.002

    authors： Ward SM,Himmelstein DS,Ren Y,Fu Y,Yu XW,Roberts K,Binder LI,Sahara N

    更新日期：2014-07-01 00:00:00

  • Imaging biomarkers of epileptogenecity after traumatic brain injury - Preclinical frontiers.

    abstract：:Posttraumatic epilepsy (PTE) is a major neurodegenerative disease accounting for 20% of symptomatic epilepsy cases. A long latent phase offers a potential window for prophylactic treatment strategies to prevent epilepsy onset, provided that the patients at risk can be identified. Some promising imaging biomarker candi...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章,评审

    doi：10.1016/j.nbd.2018.10.008

    authors： Immonen R,Harris NG,Wright D,Johnston L,Manninen E,Smith G,Paydar A,Branch C,Grohn O

    更新日期：2019-03-01 00:00:00

  • Functional genomics, genetic risk profiling and cell phenotypes in neurodegenerative disease.

    abstract：:Human genetics provides unbiased insights into the causes of human disease, which can be used to create a foundation for effective ways to more accurately diagnose patients, stratify patients for more successful clinical trials, discover and develop new therapies, and ultimately help patients choose the safest and mos...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章,评审

    doi：10.1016/j.nbd.2020.105088

    authors： Finkbeiner S

    更新日期：2020-12-01 00:00:00

  • Forebrain knock-out of torsinA reduces striatal free-water and impairs whole-brain functional connectivity in a symptomatic mouse model of DYT1 dystonia.

    abstract：:Multiple lines of evidence implicate striatal dysfunction in the pathogenesis of dystonia, including in DYT1, a common inherited form of the disease. The impact of striatal dysfunction on connected motor circuits and their interaction with other brain regions is poorly understood. Conditional knock-out (cKO) of the DY...

    journal_title：Neurobiology of disease

    pub_type： 杂志文章

    doi：10.1016/j.nbd.2017.06.015

    authors： DeSimone JC,Pappas SS,Febo M,Burciu RG,Shukla P,Colon-Perez LM,Dauer WT,Vaillancourt DE

    更新日期：2017-10-01 00:00:00