当前位置： SCI文献检索 > MOLECULAR NEUROBIOLOGY期刊下所有文献 > Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.

Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.

Abstract：

:Mutations in TAR DNA-binding protein 43 (TDP-43) are associated with familial forms of amyotrophic lateral sclerosis (ALS), while wild-type TDP-43 is a pathological hallmark of patients with sporadic ALS and frontotemporal lobar degeneration (FTLD). Various in vitro and in vivo studies have also demonstrated toxicity of both mutant and wild-type TDP-43 to neuronal cells. To study the potential additional toxicity incurred by mutant TDP-43 in vivo, we generated mutant human TDP-43 (p.M337V) transgenic mouse lines driven by the Thy-1.2 promoter (Mt-TAR) and compared them in the same experimental setting to the disease phenotype observed in wild-type TDP-43 transgenic lines (Wt-TAR) expressing comparable TDP-43 levels. Overexpression of mutant TDP-43 leads to a worsened dose-dependent disease phenotype in terms of motor dysfunction, neurodegeneration, gliosis, and development of ubiquitin and phosphorylated TDP-43 pathology. Furthermore, we show that cellular aggregate formation or accumulation of TDP-43 C-terminal fragments (CTFs) are not primarily responsible for development of the observed disease phenotype in both mutant and wild-type TDP-43 mice.

journal_name

Mol Neurobiol

journal_title

Molecular neurobiology

authors

Janssens J,Wils H,Kleinberger G,Joris G,Cuijt I,Ceuterick-de Groote C,Van Broeckhoven C,Kumar-Singh S

doi

10.1007/s12035-013-8427-5

subject

Has Abstract

pub_date

2013-08-01 00:00:00

pages

22-35

issue

1

eissn

0893-7648

issn

1559-1182

journal_volume

48

pub_type

杂志文章

相关文献

MOLECULAR NEUROBIOLOGY文献大全
  • Mouse models of polyglutamine diseases: review and data table. Part I.

    abstract：:Polyglutamine (polyQ) disorders share many similarities, such as a common mutation type in unrelated human causative genes, neurological character, and certain aspects of pathogenesis, including morphological and physiological neuronal alterations. The similarities in pathogenesis have been confirmed by findings that ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1007/s12035-012-8315-4

    authors： Figiel M,Szlachcic WJ,Switonski PM,Gabka A,Krzyzosiak WJ

    更新日期：2012-10-01 00:00:00

  • Plasmalogens Inhibit Endocytosis of Toll-like Receptor 4 to Attenuate the Inflammatory Signal in Microglial Cells.

    abstract：:Microglial activation is a pathological feature of many neurodegenerative diseases and the role of cellular lipids in these diseases is mostly unknown. It was known that the special ether lipid plasmalogens (Pls) were reduced in the brain and blood samples of Alzheimer's disease (AD) patients. It has recently been rep...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-018-1307-2

    authors： Ali F,Hossain MS,Sejimo S,Akashi K

    更新日期：2019-05-01 00:00:00

  • Brain GABAA receptors studied with subunit-specific antibodies.

    abstract：:Brain GABAA/benzodiazepine receptors are highly heterogeneous. This heterogeneity is largely derived from the existence of many pentameric combinations of at least 16 different subunits that are differentially expressed in various brain regions and cell types. This molecular heterogeneity leads to binding differences ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1007/BF02740747

    authors： De Blas AL

    更新日期：1996-02-01 00:00:00

  • Methylphenidate Causes Behavioral Impairments and Neuron and Astrocyte Loss in the Hippocampus of Juvenile Rats.

    abstract：:Although the use, and misuse, of methylphenidate is increasing in childhood and adolescence, there is little information about the consequences of this psychostimulant chronic use on brain and behavior during development. The aim of the present study was to investigate hippocampus biochemical, histochemical, and behav...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-016-9987-y

    authors： Schmitz F,Pierozan P,Rodrigues AF,Biasibetti H,Grunevald M,Pettenuzzo LF,Scaini G,Streck EL,Netto CA,Wyse ATS

    更新日期：2017-08-01 00:00:00

  • Pre-hospital Delay after Acute Ischemic Stroke in Central Urban China: Prevalence and Risk Factors.

    abstract：:Timely thrombolytic treatment is paramount after acute ischemic stroke (AIS); however, a large proportion of patients experience substantial delays in presentation to hospital. This study evaluates the prevalence and risk factors in pre-hospital delays after AIS in central urban China. AIS patients from 66 hospitals i...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-016-9750-4

    authors： Zhou Y,Yang T,Gong Y,Li W,Chen Y,Li J,Wang M,Yin X,Hu B,Lu Z

    更新日期：2017-05-01 00:00:00

  • Enhancing Endogenous Nitric Oxide by Whole Body Periodic Acceleration Elicits Neuroprotective Effects in Dystrophic Neurons.

    abstract：:We have previously shown that inadequate dystrophin in cortical neurons in mdx mice is associated with age-dependent dyshomeostasis of resting intracellular Ca2+ ([Ca2+]i) and Na+ ([Na+]i), elevated reactive oxygen species (ROS) production, increase in neuronal damage and cognitive deficit. In this study, we assessed ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-018-1018-8

    authors： Lopez JR,Uryash A,Kolster J,Estève E,Zhang R,Adams JA

    更新日期：2018-11-01 00:00:00

  • Effect of CR1 Genetic Variants on Cerebrospinal Fluid and Neuroimaging Biomarkers in Healthy, Mild Cognitive Impairment and Alzheimer's Disease Cohorts.

    abstract：:The complement component (3b/4b) receptor 1 gene (CR1) is considered as one of the most important genetic susceptibility loci in Alzheimer's disease (AD). However, to date, few studies were performed to discover the possible effect of CR1 genetic variants on AD pathology in the brain. Here, we evaluated the potential ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-015-9638-8

    authors： Zhu XC,Wang HF,Jiang T,Lu H,Tan MS,Tan CC,Tan L,Tan L,Yu JT,Alzheimer’s Disease Neuroimaging Initiative.

    更新日期：2017-01-01 00:00:00

  • Paradoxical signal transduction in neurobiological systems.

    abstract：:Information processing in neurobiological systems is commonly thought to rely on the assessment of a signal-to-noise ratio as the key mechanism of signal detection; it assumes and requires that both signal and noise are concurrently available. An alternative theory holds that detection proceeds by the system appreciat...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1385/MN:24:1-3:145

    authors： Colpaert FC,Frégnac Y

    更新日期：2001-08-01 00:00:00

  • Purmorphamine as a Shh Signaling Activator Small Molecule Promotes Motor Neuron Differentiation of Mesenchymal Stem Cells Cultured on Nanofibrous PCL Scaffold.

    abstract：:There is variety of stem cell sources but problems in ethical issues, contamination, and normal karyotype cause many limitations in obtaining and using these cells. The cells in Wharton's jelly region of umbilical cord are abundant and available stem cells with low immunological incompatibility, which could be conside...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-016-0090-1

    authors： Bahrami N,Bayat M,Mohamadnia A,Khakbiz M,Yazdankhah M,Ai J,Ebrahimi-Barough S

    更新日期：2017-09-01 00:00:00

  • Transcriptional Profiling of Individual Airway Projecting Vagal Sensory Neurons.

    abstract：:Bronchopulmonary sensory neurons are derived from the vagal sensory ganglia and are essential for monitoring the physical and chemical environment of the airways and lungs. Subtypes are heterogenous in their responsiveness to stimuli, phenotype, and developmental origin, but they collectively serve to regulate normal ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-019-01782-8

    authors： Mazzone SB,Tian L,Moe AAK,Trewella MW,Ritchie ME,McGovern AE

    更新日期：2020-02-01 00:00:00

  • Control of receptor sensitivity at the mRNA level.

    abstract：:Neurons are able to adjust the sensitivity of receptor-mediated processes according to the level of receptor activation. Extrapolating from our knowledge of other cellular proteins, regulation of receptor mRNA availability would provide a highly economical means of achieving this objective. Epidermal growth factor is ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1007/BF02769175

    authors： Morris BJ

    更新日期：1993-10-01 00:00:00

  • The Role of WD40-Repeat Protein 62 (MCPH2) in Brain Growth: Diverse Molecular and Cellular Mechanisms Required for Cortical Development.

    abstract：:Genetic disruptions of spindle/centrosome-associated WD40-repeat protein 62 (WDR62) are causative for autosomal recessive primary microcephaly (MCPH) and a broader range of cortical malformations. Since the identification of WDR62 as encoded by the MCPH2 locus in 2010, recent studies that have deleted/depleted WDR62 i...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1007/s12035-017-0778-x

    authors： Shohayeb B,Lim NR,Ho U,Xu Z,Dottori M,Quinn L,Ng DCH

    更新日期：2018-07-01 00:00:00

  • Maternal Folic Acid Supplementation During Pregnancy Improves Neurobehavioral Development in Rat Offspring.

    abstract：:Maternal folate status during pregnancy may influence central nervous system (CNS) development in offspring. However, the recommended intakes of folic acid for women of childbearing age differ among countries and there is still no consensus about whether folic acid should be supplemented continuously throughout pregna...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-017-0534-2

    authors： Wang X,Li W,Li S,Yan J,Wilson JX,Huang G

    更新日期：2018-03-01 00:00:00

  • The Functional and Molecular Properties, Physiological Functions, and Pathophysiological Roles of GluN2A in the Central Nervous System.

    abstract：:The NMDA receptor, which is heavily involved in several human brain diseases, is a heteromeric ligand-gated ion channel that interacts with multiple intracellular proteins through the C-termini of different subunits. GluN2A and GluN2B are the two primary types of GluN2 subunits in the forebrain. During the development...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1007/s12035-016-9715-7

    authors： Sun Y,Cheng X,Zhang L,Hu J,Chen Y,Zhan L,Gao Z

    更新日期：2017-03-01 00:00:00

  • Sphingosine Kinase-1 Is Essential for Maintaining External/Outer Limiting Membrane and Associated Adherens Junctions in the Aging Retina.

    abstract：:Sphingosine-1-phosphate (S1P) produced by sphingosine kinases (SPHK1 and SPHK2) is a signaling molecule involved in cell proliferation and formation of cellular junctions. In this study, we characterized the retinas of Sphk1 knockout (KO) mice by electron microscopy and immunocytochemistry. We also tested cultured Mül...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-019-1599-x

    authors： Wilkerson JL,Stiles MA,Gurley JM,Grambergs RC,Gu X,Elliott MH,Proia RL,Mandal NA

    更新日期：2019-10-01 00:00:00

  • Reduced CaM Kinase II and CaM Kinase IV Activities Underlie Cognitive Deficits in NCKX2 Heterozygous Mice.

    abstract：:Among five members of the K+-dependent Na+/Ca2+ exchanger (NCKX) family (NCKX1-5), only NCKX2 is highly expressed in mouse brain. NCKX2 in plasma membranes mediates cytosolic calcium excretion through electrogenic exchange of 4 Na+ for 1 Ca2+ and 1 K+. Here, we observed significantly decreased levels of NCKX2 protein ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-017-0596-1

    authors： Moriguchi S,Kita S,Yabuki Y,Inagaki R,Izumi H,Sasaki Y,Tagashira H,Horie K,Takeda J,Iwamoto T,Fukunaga K

    更新日期：2018-05-01 00:00:00

  • The Severity of Gliosis in Hippocampal Sclerosis Correlates with Pre-Operative Seizure Burden and Outcome After Temporal Lobectomy.

    abstract：:Astrogliosis and microgliosis in hippocampal sclerosis (HS) are widespread and are postulated to contribute to the pro-excitatory neuropathological environment. This study aimed to establish if seizure burden at the time of surgery or post-surgical outcome were correlated with the extent of gliosis in HS. As a seconda...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-015-9465-y

    authors： Johnson AM,Sugo E,Barreto D,Hiew CC,Lawson JA,Connolly AM,Somerville E,Hasic E,Bye AM,Cunningham AM

    更新日期：2016-10-01 00:00:00

  • Towards a TDP-43-Based Biomarker for ALS and FTLD.

    abstract：:TDP-43 accumulates in nerve cells of nearly all cases of amyotrophic lateral sclerosis (ALS; the commonest form of motor neuron disease) and in the majority of Tau-negative frontotemporal lobar degeneration (FTLD). There is currently no biochemical test or marker of disease activity for ALS or FTLD, and the clinical d...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1007/s12035-018-0947-6

    authors： Feneberg E,Gray E,Ansorge O,Talbot K,Turner MR

    更新日期：2018-10-01 00:00:00

  • Multi-target, neuroprotective and neurorestorative M30 improves cognitive impairment and reduces Alzheimer's-like neuropathology and age-related alterations in mice.

    abstract：:Based on a multimodal drug design strategy for age-related neurodegenerative diseases, we have synthesized a multifunctional nontoxic, brain-permeable iron-chelating compound, M30, possessing the neuroprotective N-propargyl moiety of the anti-Parkinsonian drug, monoamine oxidase-B inhibitor, rasagiline and the antioxi...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-012-8304-7

    authors： Kupershmidt L,Amit T,Bar-Am O,Weinreb O,Youdim MB

    更新日期：2012-08-01 00:00:00

  • Molecular Chaperone Hsp70 and Its Constitutively Active Form Hsc70 Play an Indispensable Role During Eye Development of Drosophila melanogaster.

    abstract：:In the present study, we demonstrate that molecular chaperone Hsp70 and Hsc70 is essential for normal organization and development of ommatidial cells in Drosophila melanogaster eye. An exogenously expressed dominant negative mutant of Hsp70 (K71E) and Hsc70.4 (K71S and D206S) in an eye-specific manner resulted in eye...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-017-0650-z

    authors： Kumar A,Tiwari AK

    更新日期：2018-05-01 00:00:00

  • Increased Neuronal Differentiation of Neural Progenitor Cells Derived from Phosphovimentin-Deficient Mice.

    abstract：:Vimentin is an intermediate filament (also known as nanofilament) protein expressed in several cell types of the central nervous system, including astrocytes and neural stem/progenitor cells. Mutation of the vimentin serine sites that are phosphorylated during mitosis (VIM SA/SA ) leads to cytokinetic failures in fibr...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-017-0759-0

    authors： Chen M,Puschmann TB,Marasek P,Inagaki M,Pekna M,Wilhelmsson U,Pekny M

    更新日期：2018-07-01 00:00:00

  • Do Human Endogenous Retroviruses Contribute to Multiple Sclerosis, and if So, How?

    abstract：:The gammaretroviral human endogenous retrovirus (HERV) families MRSV/HERV-W and HERV-H (including the closely related HERV-Fc1) are associated with an increased risk of multiple sclerosis (MS). Complete HERV sequences betray their endogenous retroviral origin, with open reading frames in gag, pro, pol and env being fl...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1007/s12035-018-1255-x

    authors： Morris G,Maes M,Murdjeva M,Puri BK

    更新日期：2019-04-01 00:00:00

  • Neurodegeneration Alters Metabolic Profile and Sirt 1 Signaling in High-Fat-Induced Obese Mice.

    abstract：:Different factors may contribute to the development of neurodegenerative diseases. Among them, metabolic syndrome (MS), which has reached epidemic proportions, has emerged as a potential element that may be involved in neurodegeneration. Furthermore, studies have shown the importance of the sirtuin family in neuronal ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-016-9927-x

    authors： Lima LCF,Saliba SW,Andrade JMO,Cunha ML,Cassini-Vieira P,Feltenberger JD,Barcelos LS,Guimarães ALS,de-Paula AMB,de Oliveira ACP,Santos SHS

    更新日期：2017-07-01 00:00:00

  • Cannabinoids and gliomas.

    abstract：:Cannabinoids, the active components of Cannabis sativa L., act in the body by mimicking endogenous substances--the endocannabinoids--that activate specific cell surface receptors. Cannabinoids exert various palliative effects in cancer patients. In addition, cannabinoids inhibit the growth of different types of tumor ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章,评审

    doi：10.1007/s12035-007-0002-5

    authors： Velasco G,Carracedo A,Blázquez C,Lorente M,Aguado T,Haro A,Sánchez C,Galve-Roperh I,Guzmán M

    更新日期：2007-08-01 00:00:00

  • Correction to: Intranasal Cotinine Plus Krill Oil Facilitates Fear Extinction, Decreases Depressive-Like Behavior, and Increases Hippocampal Calcineurin A Levels in Mice.

    abstract：:The original version of this article unfortunately contained mistake in its Funding inforation. ...

    journal_title：Molecular neurobiology

    pub_type： 已发布勘误

    doi：10.1007/s12035-018-1095-8

    authors： Alvarez-Ricartes N,Oliveros-Matus P,Mendoza C,Perez-Urrutia N,Echeverria F,Iarkov A,Barreto GE,Echeverria V

    更新日期：2018-10-01 00:00:00

  • Long Non-coding RNA MALAT1 Upregulates ZEB2 Expression to Promote Malignant Progression of Glioma by Attenuating miR-124.

    abstract：:Long non-coding RNA (lncRNA) metastasis-associated lung adenocarcinoma transcript 1 (MALAT1) has been shown to play a critical role in the development of several malignancies. However, the potential molecular mechanism of MALAT1 in glioma remains unclear. In the present study, we found that the expression of MALAT1 wa...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-020-02165-0

    authors： Cheng H,Zhao H,Xiao X,Huang Q,Zeng W,Tian B,Ma T,Lu D,Jin Y,Li Y

    更新日期：2020-10-19 00:00:00

  • Selective Aryl Hydrocarbon Receptor Modulator 3,3'-Diindolylmethane Impairs AhR and ARNT Signaling and Protects Mouse Neuronal Cells Against Hypoxia.

    abstract：:The neuroprotective potential of 3,3'-diindolylmethane (DIM), which is a selective aryl hydrocarbon receptor modulator, has recently been shown in cellular and animal models of Parkinson's disease and lipopolysaccharide-induced inflammation. However, there are no data concerning the protective capacity and mechanisms ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-015-9471-0

    authors： Rzemieniec J,Litwa E,Wnuk A,Lason W,Krzeptowski W,Kajta M

    更新日期：2016-10-01 00:00:00

  • Synthetic Prion Selection and Adaptation.

    abstract：:Prion pathologies are characterized by the conformational conversion of the cellular prion protein (PrPC) into a pathological infectious isoform, known as PrPSc. The latter acquires different abnormal conformations, which are associated with specific pathological phenotypes. Recent evidence suggests that prions adapt ...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-018-1279-2

    authors： Bistaffa E,Moda F,Virgilio T,Campagnani I,De Luca CMG,Rossi M,Salzano G,Giaccone G,Tagliavini F,Legname G

    更新日期：2019-04-01 00:00:00

  • Loss of Pericytes in Radiation Necrosis after Glioblastoma Treatments.

    abstract：:Radiation necrosis (RN) in brain tumor patients is often symptomatic, persistent without immediate resolution, and confused with tumor recurrence. Cerebral vascular pericytes are essential for endothelial function, vascular integrity, and angiogenesis. In this study, we showed that the loss of pericytes is involved in...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-017-0695-z

    authors： Lee ST,Seo Y,Bae JY,Chu K,Kim JW,Choi SH,Kim TM,Kim IH,Park SH,Park CK

    更新日期：2018-06-01 00:00:00

  • Infantile Amnesia Is Related to Developmental Immaturity of the Maintenance Mechanisms for Long-Term Potentiation.

    abstract：:Infantile amnesia (IA) refers to the inability of adults to recall episodic memories from infancy or early childhood. While several hypotheses have been proposed to explain the occurrence of IA, the neurobiological and molecular bases for this accelerated forgetting phenomenon remain elusive. Using hippocampus-depende...

    journal_title：Molecular neurobiology

    pub_type： 杂志文章

    doi：10.1007/s12035-018-1119-4

    authors： Tsai TC,Huang CC,Hsu KS

    更新日期：2019-02-01 00:00:00