Influence of ADAM10 on prion protein processing and scrapie infectiosity in vivo.

Abstract:

:Both the cellular prion protein (PrP(c)) and the amyloid precursor protein (APP) are physiologically subjected to complex proteolytic processing events. While for APP the proteinases involved--alpha-, beta- and gamma-secretase--have been identified in vitro and in vivo, the cleavage of PrP(c) by now has been linked only to the shedding activity of the metalloproteinase ADAM10 and/or ADAM17 in cell culture. Here we show that neuronal overexpression of the alpha-secretase ADAM10 in mice reduces all PrP(c) species detected in the brain instead of leading to enhanced amounts of specific cleavage products of PrP(c). Additionally, the incubation time of mice after scrapie infection is significantly increased in mice moderately overexpressing ADAM10. This indicates that overexpression of ADAM10 rather influences the amount of the cellular prion protein than its processing in vivo.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Endres K,Mitteregger G,Kojro E,Kretzschmar H,Fahrenholz F

doi

10.1016/j.nbd.2009.07.015

subject

Has Abstract

pub_date

2009-11-01 00:00:00

pages

233-41

issue

2

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(09)00175-2

journal_volume

36

pub_type

杂志文章
  • Prenatal stress elicits regionally selective changes in basal FGF-2 gene expression in adulthood and alters the adult response to acute or chronic stress.

    abstract::Exposure to stress during pregnancy influences the trajectory of brain development resulting in permanent alterations that may contribute to increased susceptibility to subsequent cognitive or neuropsychiatric disorders. In this manuscript, we examined the effects of prenatal stress on the expression of basic fibrobla...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2005.05.005

    authors: Fumagalli F,Bedogni F,Slotkin TA,Racagni G,Riva MA

    更新日期:2005-12-01 00:00:00

  • β-catenin aggregation in models of ALS motor neurons: GSK3β inhibition effect and neuronal differentiation.

    abstract::Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by motor neuron death. A 20% of familial ALS cases are associated with mutations in the gene coding for superoxide dismutase 1 (SOD1). The accumulation of abnormal aggregates of different proteins is a common feature in motor neurons of p...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2019.104497

    authors: Pinto C,Medinas DB,Fuentes-Villalobos F,Maripillán J,Castro AF,Martínez AD,Osses N,Hetz C,Henríquez JP

    更新日期:2019-10-01 00:00:00

  • Differential CSF butyrylcholinesterase levels in Alzheimer's disease patients with the ApoE epsilon4 allele, in relation to cognitive function and cerebral glucose metabolism.

    abstract::Butyrylcholinesterase (BuChE) is increased in the cerebral cortex of Alzheimer's disease (AD) patients, particularly those carrying epsilon4 allele of the apolipoprotein E gene (ApoE) and certain BuChE variants that predict increased AD risk and poor response to anticholinesterase therapy. We measured BuChE activity a...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2006.07.013

    authors: Darreh-Shori T,Brimijoin S,Kadir A,Almkvist O,Nordberg A

    更新日期:2006-11-01 00:00:00

  • Rescue of adult hippocampal neurogenesis in a mouse model of HIV neurologic disease.

    abstract::The prevalence of central nervous system (CNS) neurologic dysfunction associated with human immunodeficiency virus (HIV) infection continues to increase, despite the use of antiretroviral therapy. Previous work has focused on the deleterious effects of HIV on mature neurons and on development of neuroprotective strate...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2010.12.002

    authors: Lee MH,Wang T,Jang MH,Steiner J,Haughey N,Ming GL,Song H,Nath A,Venkatesan A

    更新日期:2011-03-01 00:00:00

  • Particular vulnerability of rat mesencephalic dopaminergic neurons to tetrahydrobiopterin: Relevance to Parkinson's disease.

    abstract::We determined whether tetrahydrobiopterin(BH4), an endogenous cofactor for dopamine(DA) synthesis, causes preferential damage to DArgic neurons among primary cultured rat mesencephalic neurons and whether the death mechanism has relevance to Parkinson's disease (PD). DArgic neurons were more vulnerable to BH4 than non...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2006.08.024

    authors: Lee SY,Moon Y,Hee Choi D,Jin Choi H,Hwang O

    更新日期:2007-01-01 00:00:00

  • Identification and functional dissection of localization signals within ataxin-3.

    abstract::Spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD) belongs to a group of autosomal dominant neurodegenerative diseases, which are caused by the expansion of a polyglutamine repeat in the affected protein, in this case ataxin-3. Ataxin-3 is mainly localized in the cytoplasm; however, one hallmark of S...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2009.07.020

    authors: Antony PM,Mäntele S,Mollenkopf P,Boy J,Kehlenbach RH,Riess O,Schmidt T

    更新日期:2009-11-01 00:00:00

  • Prolonged prophylactic effects of gabapentin on status epilepticus-induced neocortical injury.

    abstract::Long-term consequences of status epilepticus (SE) occur in a significant proportion of those who survive the acute episode. We developed an in vivo model of acute focal neocortical SE (FSE) to study long-term effects on local cortical structure and function and potential strategies to mitigate adverse consequences of ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2020.104949

    authors: Perez-Ramirez MB,Gu F,Prince DA

    更新日期:2020-08-01 00:00:00

  • Interneuronal calcium channel abnormalities in posttraumatic epileptogenic neocortex.

    abstract::Decreased release probability (Pr) and increased failure rate for monosynaptic inhibitory postsynaptic currents (IPSCs) indicate abnormalities in presynaptic inhibitory terminals on pyramidal (Pyr) neurons of the undercut (UC) model of posttraumatic epileptogenesis. These indices of inhibition are normalized in high [...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2011.11.006

    authors: Faria LC,Parada I,Prince DA

    更新日期:2012-02-01 00:00:00

  • Death of motoneurons induced by trophic deprivation or by excitotoxicity is not prevented by overexpression of SMN.

    abstract::The telomeric copy of the survival motor neuron gene (SMN1) is deleted or mutated in all spinal muscular atrophy (SMA) patients and these patients present mainly a loss in spinal motoneurons. Although studies performed in HeLa cells suggest that SMN may be involved in the biogenesis and possibly in recycling of splice...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2000.0374

    authors: Cisterni C,Kallenbach S,Jordier F,Bagnis C,Pettmann B

    更新日期:2001-04-01 00:00:00

  • Neutralizing anti-interleukin-1β antibodies modulate fetal blood-brain barrier function after ischemia.

    abstract::We have previously shown that increases in blood-brain barrier permeability represent an important component of ischemia-reperfusion related brain injury in the fetus. Pro-inflammatory cytokines could contribute to these abnormalities in blood-brain barrier function. We have generated pharmacological quantities of mou...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2014.09.007

    authors: Chen X,Sadowska GB,Zhang J,Kim JE,Cummings EE,Bodge CA,Lim YP,Makeyev O,Besio WG,Gaitanis J,Threlkeld SW,Banks WA,Stonestreet BS

    更新日期:2015-01-01 00:00:00

  • Putative glucosensing property in rat and human activated microglia.

    abstract::Microglial cells involved in the pathogenesis of many neurodegenerative diseases acquire the features of cytotoxic and phagocytic cells in response to certain pathogens and inflammatory signals. K(ATP) channels are energy sensors of ATP availability that link the cell's metabolic state to its membrane excitability. In...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2003.11.019

    authors: Ramonet D,Rodríguez MJ,Pugliese M,Mahy N

    更新日期:2004-10-01 00:00:00

  • Ca2+/calmodulin-dependent modulation of cell cycle elements pRb and p27kip1 involved in the enhanced proliferation of lymphoblasts from patients with Alzheimer dementia.

    abstract::Failure of cell cycle regulation in neurons might be critically involved in the process of neurodegeneration in Alzheimer's disease (AD). We present here evidence to support the hypothesis that cell cycle alterations occur in cells other than neurons in AD sufferers. Lymphocytes from AD patients immortalized with Epst...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/s0969-9961(03)00040-8

    authors: de las Cuevas N,Urcelay E,Hermida OG,Saíz-Diaz RA,Bermejo F,Ayuso MS,Martín-Requero A

    更新日期:2003-08-01 00:00:00

  • Genetically determined susceptibility to neurodegeneration is associated with expression of inflammatory genes.

    abstract::Axonal damage, a core feature of neurological diseases, induces a retrograde reaction in neurons and surrounding glia. We determined transcriptional profiles of this reaction using Affymetrix oligonucleotide arrays. Gene expression was examined in spinal cord tissue prior to injury and following ventral root avulsion ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2006.05.016

    authors: Swanberg M,Duvefelt K,Diez M,Hillert J,Olsson T,Piehl F,Lidman O

    更新日期:2006-10-01 00:00:00

  • Jmjd3 mediates blood-spinal cord barrier disruption after spinal cord injury by regulating MMP-3 and MMP-9 expressions.

    abstract::The disruption of the blood-spinal cord barrier (BSCB) by matrix metalloprotease (MMP) activation is a detrimental event that leads to blood cell infiltration, inflammation, and apoptosis, thereby contributing to permanent neurological disability after spinal cord injury (SCI). However, the molecular mechanisms underl...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2016.07.015

    authors: Lee JY,Na WH,Choi HY,Lee KH,Ju BG,Yune TY

    更新日期:2016-11-01 00:00:00

  • Overexpression of wild-type human APP in mice causes cognitive deficits and pathological features unrelated to Abeta levels.

    abstract::Transgenic mice expressing mutant human amyloid precursor protein (APP) develop an age-dependent amyloid pathology and memory deficits, but no overt neuronal loss. Here, in mice overexpressing wild-type human APP (hAPP(wt)) we found an early memory impairment, particularly in the water maze and to a lesser extent in t...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2008.11.005

    authors: Simón AM,Schiapparelli L,Salazar-Colocho P,Cuadrado-Tejedor M,Escribano L,López de Maturana R,Del Río J,Pérez-Mediavilla A,Frechilla D

    更新日期:2009-03-01 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

  • Exacerbation of ischemic brain injury in hypercholesterolemic mice is associated with pronounced changes in peripheral and cerebral immune responses.

    abstract::Inflammation contributes to ischemic brain injury. However, translation of experimental findings from animal models into clinical trials is still ineffective, since the majority of human stroke studies mainly focus on acute neuroprotection, thereby neglecting inflammatory mechanisms and inflammation-associated co-morb...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2013.10.022

    authors: Herz J,Hagen SI,Bergmüller E,Sabellek P,Göthert JR,Buer J,Hansen W,Hermann DM,Doeppner TR

    更新日期:2014-02-01 00:00:00

  • Pmp22 mutant allele-specific siRNA alleviates demyelinating neuropathic phenotype in vivo.

    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

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2017.01.006

    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

  • Neurodegenerative disorders: insights from the nematode Caenorhabditis elegans.

    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

    pub_type: 杂志文章,评审

    doi:10.1016/j.nbd.2010.05.012

    authors: Dimitriadi M,Hart AC

    更新日期:2010-10-01 00:00:00

  • Rescue of Fmr1KO phenotypes with mGluR5 inhibitors: MRZ-8456 versus AFQ-056.

    abstract::Metabotropic glutamate receptor 5 (mGluR5) is a drug target for central nervous system disorders such as fragile X syndrome that involve excessive glutamate-induced excitation. We tested the efficacy of a novel negative allosteric modulator of mGluR5 developed by Merz Pharmaceuticals, MRZ-8456, in comparison to MPEP a...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2018.08.008

    authors: Westmark PR,Dekundy A,Gravius A,Danysz W,Westmark CJ

    更新日期:2018-11-01 00:00:00

  • Effects of environmental manipulations in genetically targeted animal models of affective disorders.

    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

    pub_type: 杂志文章,评审

    doi:10.1016/j.nbd.2012.04.003

    authors: Renoir T,Pang TY,Hannan AJ

    更新日期:2013-09-01 00:00:00

  • Purkinje cell-specific Grip1/2 knockout mice show increased repetitive self-grooming and enhanced mGluR5 signaling in cerebellum.

    abstract::Cerebellar Purkinje cell (PC) loss is a consistent pathological finding in autism. However, neural mechanisms of PC-dysfunction in autism remain poorly characterized. Glutamate receptor interacting proteins 1/2 (Grip1/2) regulate AMPA receptor (AMPAR) trafficking and synaptic strength. To evaluate role of PC-AMPAR sig...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2019.104602

    authors: Mejias R,Chiu SL,Han M,Rose R,Gil-Infante A,Zhao Y,Huganir RL,Wang T

    更新日期:2019-12-01 00:00:00

  • Epigenetic mechanisms underlying human epileptic disorders and the process of epileptogenesis.

    abstract::The rapidly emerging science of epigenetics and epigenomic medicine promises to reveal novel insights into the susceptibility to and the onset and progression of epileptic disorders. Epigenetic regulatory mechanisms are now implicated in orchestrating aspects of neural development (e.g., cell fate specification and ma...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1016/j.nbd.2010.02.005

    authors: Qureshi IA,Mehler MF

    更新日期:2010-07-01 00:00:00

  • Cerebrovascular pathology during the progression of experimental Alzheimer's disease.

    abstract::Clinical and experimental evidence point to a possible role of cerebrovascular dysfunction in Alzheimer's disease (AD). The 5xFAD mouse model of AD expresses human amyloid precursor protein and presenilin genes with mutations found in AD patients. It remains unknown whether amyloid deposition driven by these mutations...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2016.01.001

    authors: Giannoni P,Arango-Lievano M,Neves ID,Rousset MC,Baranger K,Rivera S,Jeanneteau F,Claeysen S,Marchi N

    更新日期:2016-04-01 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

  • Bax deletion does not protect neurons from BSE-induced death.

    abstract::Neurodegeneration is a common neuropathological feature of prion diseases. Although evidence of apoptosis was found in natural and experimental prion diseases, the precise mechanisms by which neurons die are poorly understood. The pro-apoptotic BAX protein, a key factor of the mitochondrial pathway, plays a central ro...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2006.05.013

    authors: Coulpier M,Messiaen S,Hamel R,Fernández de Marco M,Lilin T,Eloit M

    更新日期:2006-09-01 00:00:00

  • Neuronal tissue-specific ribonucleoprotein complex formation on SOD1 mRNA: alterations by ALS SOD1 mutations.

    abstract::Amyotrophic lateral sclerosis (ALS) is a fatal disease of unknown etiology. Mutations in copper/zinc superoxide dismutase (SOD1) are the most commonly associated genetic abnormality. Given that SOD1 is ubiquitously expressed, the exclusive vulnerability of motor neurons is one of the most puzzling issues in ALS resear...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2006.03.007

    authors: Ge WW,Leystra-Lantz C,Sanelli TR,McLean J,Wen W,Strong W,Strong MJ

    更新日期:2006-08-01 00:00:00

  • Alzheimer pathology disorganizes cortico-cortical circuitry: direct evidence from a transgenic animal model.

    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

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2004.01.008

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

    更新日期:2004-06-01 00:00:00

  • Leptin enhances adult neurogenesis and reduces pathological features in a transgenic mouse model of Alzheimer's disease.

    abstract::Alzheimer's disease (AD) is the most common dementia worldwide and is characterized by the presence of senile plaques by amyloid-beta (Aβ) and neurofibrillary tangles of hyperphosphorylated Tau protein. These changes lead to progressive neuronal degeneration and dysfunction, resulting in severe brain atrophy and cogni...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2020.105219

    authors: Calió ML,Mosini AC,Marinho DS,Salles GN,Massinhani FH,Ko GM,Porcionatto MA

    更新日期:2021-01-01 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