How the shapes of seeds can influence pathology.

Abstract:

:It is widely accepted that the loss of function of different cellular proteins following their aggregation into highly stable aggregates or the gain of pathologic function of the resulting macromolecular assemblies or both processes are tightly associated to distinct debilitating neurodegenerative diseases such as Alzheimer's, Parkinson's, Creutzfeldt-Jacob, Amyotrophic Lateral Sclerosis and Huntington's diseases. How the aggregation of one given protein leads to distinct diseases is unclear. Here, a structural-molecular explanation based on the ability of proteins such as α-synuclein or tau to form assemblies that differ by their intrinsic architecture, stability, seeding capacity, and surfaces is proposed to account for distinct synucleinopathies and tauopathies. The shape and surfaces of the seeds is proposed to define at the same time their seeding capacity, interactome and tropism for defined neuronal cells within the central nervous system.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Melki R

doi

10.1016/j.nbd.2017.03.011

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

201-208

issue

Pt B

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(17)30060-8

journal_volume

109

pub_type

杂志文章
  • Locomotor hyperactivity and alterations in dopamine neurotransmission are associated with overexpression of A53T mutant human alpha-synuclein in mice.

    abstract::Genetic and biochemical abnormalities associated with alpha-synuclein are implicated in the etiology of Parkinson's disease (PD). In this study, altered locomotor behavior linked to the expression of mutant or wildtype human alpha-synuclein was investigated. A53T alpha-synuclein transgenic (A53T-tg) mice exhibited nor...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2005.08.005

    authors: Unger EL,Eve DJ,Perez XA,Reichenbach DK,Xu Y,Lee MK,Andrews AM

    更新日期:2006-02-01 00:00:00

  • Expression of the methyl-CpG-binding protein MeCP2 in rat brain. An ontogenetic study.

    abstract::Rett syndrome (RS) is caused by mutations in the gene encoding methyl-CpG-binding protein 2 (MeCP2) and is characterized by arrested postnatal neurodevelopment. We followed the expression of MeCP2 protein in various brain structures of normal rat from birth to 2 years of age. By measuring the amount of protein using t...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2003.10.011

    authors: Cassel S,Revel MO,Kelche C,Zwiller J

    更新日期:2004-03-01 00:00:00

  • Activation of caspase-1 dependent interleukins in developmental brain trauma.

    abstract::Focal mechanical cortical trauma triggers diffuse apoptotic neurodegeneration in the developing rat brain which is associated with invasion of brain tissue with inflammatory mediators. We hypothesized that caspase-1 and the two caspase-1-processed cytokines, interleukin (IL)-1beta and IL-18, are involved in trauma-ind...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2006.11.003

    authors: Sifringer M,Stefovska V,Endesfelder S,Stahel PF,Genz K,Dzietko M,Ikonomidou C,Felderhoff-Mueser U

    更新日期:2007-03-01 00:00:00

  • Reduced dopamine transporter density in the ventral striatum of patients with Parkinson's disease and pathological gambling.

    abstract::Pathological gambling (PG) represents a behavioral side effect of dopamine replacement therapy in a minority of patients with Parkinson's disease (PD). Using striatal dopamine transporter (DAT) with single photon emission tomography we assessed presynaptic dopaminergic function in 8 PD patients with PG, 21 matched PD ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2010.03.013

    authors: Cilia R,Ko JH,Cho SS,van Eimeren T,Marotta G,Pellecchia G,Pezzoli G,Antonini A,Strafella AP

    更新日期:2010-07-01 00:00:00

  • Proteomic signatures of brain regions affected by tau pathology in early and late stages of Alzheimer's disease.

    abstract:BACKGROUND:Alzheimer's disease (AD) is the most common neurodegenerative disorder. Depositions of amyloid β peptide (Aβ) and tau protein are among the major pathological hallmarks of AD. Aβ and tau burden follows predictable spatial patterns during the progression of AD. Nevertheless, it remains obscure why certain bra...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2019.104509

    authors: Mendonça CF,Kuras M,Nogueira FCS,Plá I,Hortobágyi T,Csiba L,Palkovits M,Renner É,Döme P,Marko-Varga G,Domont GB,Rezeli M

    更新日期:2019-10-01 00:00:00

  • Dehydroepiandrosterone reduces expression and activity of BACE in NT2 neurons exposed to oxidative stress.

    abstract::Recently, we showed that oxidative stress activates the expression and activity of the beta-site AbetaPP-cleaving enzyme (BACE), an aspartyl protease responsible for the beta-secretase cleavage of AbetaPP. The identification of compounds able to prevent the induction of this event is an important goal of therapeutic s...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/s0969-9961(03)00131-1

    authors: Tamagno E,Guglielmotto M,Bardini P,Santoro G,Davit A,Di Simone D,Danni O,Tabaton M

    更新日期:2003-11-01 00:00:00

  • Cholesterol retention in Alzheimer's brain is responsible for high beta- and gamma-secretase activities and Abeta production.

    abstract::Alzheimer's disease (AD) is characterized by overproduction of A beta derived from APP cleavage via beta- and gamma-secretase pathway. Recent evidence has linked altered cholesterol metabolism to AD pathogenesis. In this study, we show that AD brain had significant cholesterol retention and high beta- and gamma-secret...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2007.10.005

    authors: Xiong H,Callaghan D,Jones A,Walker DG,Lue LF,Beach TG,Sue LI,Woulfe J,Xu H,Stanimirovic DB,Zhang W

    更新日期:2008-03-01 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

  • Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction.

    abstract::Mutations in the gene encoding the RNA-binding protein TDP-43 cause amyotrophic lateral sclerosis (ALS), clinically and pathologically indistinguishable from the majority of 'sporadic' cases of ALS, establishing altered TDP-43 function and distribution as a primary mechanism of neurodegeneration. Transgenic mouse mode...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2018.09.024

    authors: Gordon D,Dafinca R,Scaber J,Alegre-Abarrategui J,Farrimond L,Scott C,Biggs D,Kent L,Oliver PL,Davies B,Ansorge O,Wade-Martins R,Talbot K

    更新日期:2019-01-01 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

  • Inhibition of tumor necrosis factor-alpha signaling prevents human immunodeficiency virus-1 protein Tat and methamphetamine interaction.

    abstract::Our previous studies demonstrated that the psychostimulant methamphetamine (MA) and the human immunodeficiency virus-1 (HIV-1) protein Tat interacted to cause enhanced dopaminergic neurotoxicity. The present study examined whether tumor necrosis factor-alpha (TNF-alpha) mediates the interaction between Tat and MA. In ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2006.05.005

    authors: Theodore S,Cass WA,Nath A,Steiner J,Young K,Maragos WF

    更新日期:2006-09-01 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

  • Neuroprotective effects of the Sigma-1 receptor (S1R) agonist PRE-084, in a mouse model of motor neuron disease not linked to SOD1 mutation.

    abstract::The identification of novel molecular targets crucially involved in motor neuron degeneration/survival is a necessary step for the development of hopefully more effective therapeutic strategies for amyotrophic lateral sclerosis (ALS) patients. In this view, S1R, an endoplasmic reticulum (ER)-resident receptor with cha...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2013.10.010

    authors: Peviani M,Salvaneschi E,Bontempi L,Petese A,Manzo A,Rossi D,Salmona M,Collina S,Bigini P,Curti D

    更新日期:2014-02-01 00:00:00

  • Neurostimulation techniques to enhance sleep and improve cognition in aging.

    abstract::Sleep plays a critical role in the process of memory consolidation. In particular, during non-rapid eye movement (NREM) slow wave sleep, slow-oscillations, spindles, hippocampal sharp wave ripples, and their phase coupling are involved in the process of transferring and consolidating information recently encoded and t...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1016/j.nbd.2020.104865

    authors: Grimaldi D,Papalambros NA,Zee PC,Malkani RG

    更新日期:2020-07-01 00:00:00

  • Functional MAPT haplotypes: bridging the gap between genotype and neuropathology.

    abstract::The microtubule-associated protein tau (MAPT) locus has long been associated with sporadic neurodegenerative disease, notably progressive supranuclear palsy and corticobasal degeneration, and more recently with Alzheimer's disease and Parkinson's disease. However, the functional biological mechanisms behind the geneti...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1016/j.nbd.2007.04.006

    authors: Caffrey TM,Wade-Martins R

    更新日期:2007-07-01 00:00:00

  • Truncated wild-type SOD1 and FALS-linked mutant SOD1 cause neural cell death in the chick embryo spinal cord.

    abstract::Approximately 10% of amyotrophic lateral sclerosis (ALS) cases are familial (FALS), and approximately 25% of FALS cases are caused by mutations in superoxide dismutase-1 (SOD1). Mutant (MT) SOD1 kills motor neurons because of the mutant protein's toxicity; however, the basis for toxicity is unknown. We electroporated ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2005.07.006

    authors: Ghadge GD,Wang L,Sharma K,Monti AL,Bindokas V,Stevens FJ,Roos RP

    更新日期:2006-01-01 00:00:00

  • BDNF regulates BIM expression levels in 3-nitropropionic acid-treated cortical neurons.

    abstract::3-Nitropropionic acid (3-NP) is an irreversible inhibitor of succinate dehydrogenase that has been used to explore the primary mechanisms of cell death associated with mitochondrial dysfunction and neurodegeneration in Huntington's disease. In this study we investigated the ability of brain-derived neurotrophic factor...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2009.06.006

    authors: Almeida S,Laço M,Cunha-Oliveira T,Oliveira CR,Rego AC

    更新日期:2009-09-01 00:00:00

  • Subtle microstructural changes of the striatum in a DYT1 knock-in mouse model of dystonia.

    abstract::The dystonias are comprised of a group of disorders that share common neurological abnormalities of involuntary twisting or repetitive movements and postures. The most common inherited primary dystonia is DYT1 dystonia, which is due to loss of a GAG codon in the TOR1A gene that encodes torsinA. Autopsy studies of brai...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2013.01.008

    authors: Song CH,Bernhard D,Bolarinwa C,Hess EJ,Smith Y,Jinnah HA

    更新日期:2013-06-01 00:00:00

  • Does gene deletion of AMPA GluA1 phenocopy features of schizoaffective disorder?

    abstract::Glutamatergic dysfunction is strongly implicated in schizophrenia and mood disorders. GluA1 knockout (KO) mice display schizophrenia- and depression-related abnormalities. Here, we asked whether GluA1 KO show mania-related abnormalities. KO were tested for behavior in approach/avoid conflict tests, responses to repeat...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2010.08.005

    authors: Fitzgerald PJ,Barkus C,Feyder M,Wiedholz LM,Chen YC,Karlsson RM,Machado-Vieira R,Graybeal C,Sharp T,Zarate C,Harvey-White J,Du J,Sprengel R,Gass P,Bannerman D,Holmes A

    更新日期:2010-12-01 00:00:00

  • AT2-receptor stimulation enhances axonal plasticity after spinal cord injury by upregulating BDNF expression.

    abstract::It is widely accepted that the angiotensin AT2-receptor (AT2R) has neuroprotective features. In the present study we tested pharmacological AT2R-stimulation as a therapeutic approach in a model of spinal cord compression injury (SCI) in mice using the novel non-peptide AT2R-agonist, Compound 21 (C21). Complementary ex...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2012.11.008

    authors: Namsolleck P,Boato F,Schwengel K,Paulis L,Matho KS,Geurts N,Thöne-Reineke C,Lucht K,Seidel K,Hallberg A,Dahlöf B,Unger T,Hendrix S,Steckelings UM

    更新日期:2013-03-01 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

  • Quantitative ultrasound and apoptotic death in the neonatal primate brain.

    abstract::Apoptosis is triggered in the developing mammalian brain by sedative, anesthetic or antiepileptic drugs during late gestation and early life. Whether human children are vulnerable to this toxicity mechanism remains unknown, as there are no imaging techniques to capture it. Apoptosis is characterized by distinct struct...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2019.03.032

    authors: Rosado-Mendez IM,Noguchi KK,Castañeda-Martinez L,Kirvassilis G,Wang SH,Manzella F,Swiney BS,Masuoka K,Capuano S III2,Brunner KG,Crosno K,Guerrero QW,Whitson H,Brambrink A,Simmons HS,Mejia AF,Zagzebski JA,Hall TJ,Ikono

    更新日期:2019-07-01 00:00:00

  • The epitranscriptome in stem cell biology and neural development.

    abstract::The blossoming field of epitranscriptomics has recently garnered attention across many fields by findings that chemical modifications on RNA have immense biological consequences. Methylation of nucleotides in RNA, including N6-methyladenosine (m6A), 2-O-dimethyladenosine (m6Am), N1-methyladenosine (m1A), 5-methylcytos...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1016/j.nbd.2020.105139

    authors: Vissers C,Sinha A,Ming GL,Song H

    更新日期:2020-12-01 00:00:00

  • A human single-chain Fv intrabody preferentially targets amino-terminal Huntingtin's fragments in striatal models of Huntington's disease.

    abstract::Amino-terminal fragments of huntingtin (htt) appear to result from proteolytic processing of the full-length protein in Huntington's disease (HD), and fragments containing pathological expansions of polyglutamine elicit toxicity in model systems. Such fragments are sequestered into insoluble aggregates, which may init...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2004.11.003

    authors: Miller TW,Zhou C,Gines S,MacDonald ME,Mazarakis ND,Bates GP,Huston JS,Messer A

    更新日期:2005-06-01 00:00:00

  • Triptolide protects dopaminergic neurons from inflammation-mediated damage induced by lipopolysaccharide intranigral injection.

    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

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2004.12.005

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

    更新日期:2005-04-01 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

  • The mismatch repair system protects against intergenerational GAA repeat instability in a Friedreich ataxia mouse model.

    abstract::Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder caused by a dynamic GAA repeat expansion mutation within intron 1 of the FXN gene. Studies of mouse models for other trinucleotide repeat (TNR) disorders have revealed an important role of mismatch repair (MMR) proteins in TNR instability. T...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2012.01.002

    authors: Ezzatizadeh V,Pinto RM,Sandi C,Sandi M,Al-Mahdawi S,Te Riele H,Pook MA

    更新日期:2012-04-01 00:00:00

  • Inhibition of prolyl hydroxylases by dimethyloxaloylglycine after stroke reduces ischemic brain injury and requires hypoxia inducible factor-1α.

    abstract::Pathological oxygen deprivation inhibits prolyl hydroxylase (PHD) activity and stimulates a protective cellular oxygen-sensing response in part through the stabilization and activation of the Hypoxia Inducible Factor (HIF) 1α transcription factor. The present investigation tested the therapeutic potential of enhanced ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2011.10.020

    authors: Ogle ME,Gu X,Espinera AR,Wei L

    更新日期:2012-02-01 00:00:00

  • MiR-146a promotes oligodendrocyte progenitor cell differentiation and enhances remyelination in a model of experimental autoimmune encephalomyelitis.

    abstract::The death of mature oligodendrocytes (OLs) leads to demyelination in the central nervous system (CNS) and subsequently to functional deficits. Remyelination requires the differentiation of oligodendrocyte progenitor cells (OPCs) into myelinating OLs, which in the CNS with neurodegenerative diseases such as multiple sc...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/j.nbd.2019.01.019

    authors: Zhang J,Zhang ZG,Lu M,Zhang Y,Shang X,Chopp M

    更新日期:2019-05-01 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