Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease: A cross-validation study in Q175 knock-in mice.

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：:Human immunodeficiency virus (HIV) infection continues to rise in drug-abusing populations and causes a dementing illness in a subset of individuals. Factors contributing to the development of dementia in this population remain unknown. We found that HIV-infected individuals with the E4 allele of Apolipoprotein E (Apo...

journal_title：Neurobiology of disease

authors： Turchan-Cholewo J,Liu Y,Gartner S,Reid R,Jie C,Peng X,Chen KC,Chauhan A,Haughey N,Cutler R,Mattson MP,Pardo C,Conant K,Sacktor N,McArthur JC,Hauser KF,Gairola C,Nath A

更新日期：2006-07-01 00:00:00

abstract：:We identify an essential role for the RGD (Arg-Gly-Asp tripeptide) moiety in vivo during adult peripheral neuron regenerative growth. Beyond a peripheral nerve transection there were rises in the fibronectin extracellular matrix, and striking rises in the mRNA and protein expression of integrin subunits sensitive to R...

journal_title：Neurobiology of disease

authors： Liu WQ,Martinez JA,Durand J,Wildering W,Zochodne DW

更新日期：2009-04-01 00:00:00

abstract：:Prevalent in approximately 20% of the worldwide human population, the rs6265 (also called 'Val66Met') single nucleotide polymorphism (SNP) in the gene for brain-derived neurotrophic factor (BDNF) is a common genetic variant that can alter therapeutic responses in individuals with Parkinson's disease (PD). Possession o...

journal_title：Neurobiology of disease

authors： Mercado NM,Stancati JA,Sortwell CE,Mueller RL,Boezwinkle SA,Duffy MF,Fischer DL,Sandoval IM,Manfredsson FP,Collier TJ,Steece-Collier K

更新日期：2021-01-01 00:00:00

abstract：:Recently an aspartyl protease with beta-secretase activity called BACE was identified. In the present paper we showed that BACE is modulated by the oxidative stress product 4-hydroxynonenal (HNE). Exposure of NT(2) neurons to the two classical pro-oxidant stimuli ascorbate/FeSO(4) and H(2)O(2)/FeSO(4) resulted in a si...

journal_title：Neurobiology of disease

authors： Tamagno E,Bardini P,Obbili A,Vitali A,Borghi R,Zaccheo D,Pronzato MA,Danni O,Smith MA,Perry G,Tabaton M

更新日期：2002-08-01 00:00:00

abstract：:Multiple sclerosis (MS) is the prototypical inflammatory disease of the central nervous system (CNS), leading to multifocal demyelination and neurodegeneration. The etiology of this incurable disease is unknown and remains a matter of intensive research. The possibility that microbial infections, such as viruses or ba...

journal_title：Neurobiology of disease

authors： Alonso R,Fernández-Fernández AM,Pisa D,Carrasco L

更新日期：2018-09-01 00:00:00

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

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

abstract：:Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by an expansion of a CAG repeat in the huntingtin (htt) gene, which results in an aberrant form of the protein (mhtt). This leads to motor and cognitive deficits associated with corticostriatal and hippocampal alterations. The levels of STriat...

journal_title：Neurobiology of disease

authors： García-Forn M,Martínez-Torres S,García-Díaz Barriga G,Alberch J,Milà M,Azkona G,Pérez-Navarro E

更新日期：2018-12-01 00:00:00

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

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

更新日期：2004-03-01 00:00:00

abstract：:Inhibition of mitochondrial axonal trafficking by amyloid beta (Aβ) peptides has been implicated in early pathophysiology of Alzheimer's Disease (AD). Yet, it remains unclear whether the loss of motility inevitably induces the loss of mitochondrial function, and whether restoration of axonal trafficking represents a v...

journal_title：Neurobiology of disease

authors： Zhang L,Trushin S,Christensen TA,Tripathi U,Hong C,Geroux RE,Howell KG,Poduslo JF,Trushina E

更新日期：2018-06-01 00:00:00

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

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

abstract：:Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder of children, characterized by selective death of neocortical neurons. To understand early disease mechanisms in INCL, we have studied Ppt1(Deltaex4) knock-out mouse neurons in culture and acute brain slices. Global transcript profil...

journal_title：Neurobiology of disease

authors： Ahtiainen L,Kolikova J,Mutka AL,Luiro K,Gentile M,Ikonen E,Khiroug L,Jalanko A,Kopra O

更新日期：2007-10-01 00:00:00

abstract：:Ubiquitination, a fundamental post-translational modification of intracellular proteins, is enzymatically reversed by deubiquitinase enzymes (deubiquitinases). >90 deubiquitinases have been identified. One of these enzymes, YOD1, possesses deubiquitinase activity and is similar to ovarian tumor domain-containing prote...

journal_title：Neurobiology of disease

authors： Tanji K,Mori F,Miki Y,Utsumi J,Sasaki H,Kakita A,Takahashi H,Wakabayashi K

更新日期：2018-04-01 00:00:00

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

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

abstract：:Mutations in the gene for DJ-1 have been associated with early-onset autosomal recessive parkinsonism. Previous studies of null DJ-1 mice have shown alterations in striatal dopamine (DA) transmission with no DAergic cell loss. Here we characterize a new line of DJ-1-deficient mice. A subtle locomotor deficit was prese...

journal_title：Neurobiology of disease

authors： Manning-Boğ AB,Caudle WM,Perez XA,Reaney SH,Paletzki R,Isla MZ,Chou VP,McCormack AL,Miller GW,Langston JW,Gerfen CR,Dimonte DA

更新日期：2007-08-01 00:00:00

abstract：:The impact of Abeta deposition upon cholinergic intrinsic cortical and striatal, as well as basal forebrain long projection neuronal systems was qualitatively and quantitatively evaluated in young (2-6 months) and middle-aged (10-16 months) APPswe/PS1DeltaE9 transgenic (tg) mice. Cholinergic neuritic swellings occurre...

journal_title：Neurobiology of disease

authors： Perez SE,Dar S,Ikonomovic MD,DeKosky ST,Mufson EJ

更新日期：2007-10-01 00:00:00

abstract：:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by an excessive expansion of a CAG trinucleotide repeat in the gene encoding the protein huntingtin, resulting in an elongated stretch of glutamines near the N-terminus of the protein. Here we report the derivation of a collection of ...

journal_title：Neurobiology of disease

authors： Castiglioni V,Onorati M,Rochon C,Cattaneo E

更新日期：2012-04-01 00:00:00

abstract：:Parkinson's disease (PD), characterized by the loss of dopaminergic nigrostriatal projections, is a debilitating neurodegenerative disease which produces bradykinesia, rigidity, tremor and postural instability. The dopamine precursor levodopa (L-Dopa) is the most effective treatment for the amelioration of PD signs an...

journal_title：Neurobiology of disease

authors： Prescott IA,Liu LD,Dostrovsky JO,Hodaie M,Lozano AM,Hutchison WD

更新日期：2014-11-01 00:00:00

abstract：:Cerebral ischemia activates endogenous neurogenesis in the subventricular zone (SVZ) and the dentate gyrus. Consecutively, SVZ-derived neural precursors migrate towards ischemic lesions. However, functional relevance of activated neurogenesis is limited by poor survival of new-born precursors. We therefore employed th...

journal_title：Neurobiology of disease

authors： Doeppner TR,Dietz GP,El Aanbouri M,Gerber J,Witte OW,Bähr M,Weise J

更新日期：2009-04-01 00:00:00

abstract：:Peripherin is a type III intermediate filament protein normally undetectable in most brain neurons. Here, we report a similar pattern of peripherin expression in the brains of both mice treated with systemic injections of kainic acid (KA) and in peripherin transgenic mice (Per mice) over-expressing the normal peripher...

journal_title：Neurobiology of disease

authors： Kriz J,Beaulieu JM,Julien JP,Krnjević K

更新日期：2005-03-01 00:00:00

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

authors： Caffrey TM,Wade-Martins R

更新日期：2007-07-01 00:00:00

abstract：:Type 2 diabetes (T2D) is a major risk factor for late-onset Alzheimer's disease (AD). A variety of metabolic changes related to T2D (e.g. hyperinsulinemia, hyperglycemia, and elevated branched-chain amino acids) have been proposed as mechanistic links, but the basis for this association remains unknown. Retromer-depen...

journal_title：Neurobiology of disease

authors： Morabito MV,Berman DE,Schneider RT,Zhang Y,Leibel RL,Small SA

更新日期：2014-05-01 00:00:00

abstract：:Increasing evidence has linked inflammatory processes to neurodegenerative disorders, including Alzheimer's and Parkinson's disease (PD). Tumor necrosis factor alpha (TNF-alpha) is a key inflammatory cytokine and several studies linked increased TNF-alpha to dopaminergic cell death in PD. The TNF-alpha promoter sequen...

journal_title：Neurobiology of disease

authors： Pieper HC,Evert BO,Kaut O,Riederer PF,Waha A,Wüllner U

更新日期：2008-12-01 00:00:00

abstract：:Gephyrin is a postsynaptic scaffolding protein, essential for the clustering of glycine and γ-aminobutyric acid type-A receptors (GABAARs) at inhibitory synapses. An impairment of GABAergic synaptic inhibition represents a key pathway of epileptogenesis. Recently, exonic microdeletions in the gephyrin (GPHN) gene have...

journal_title：Neurobiology of disease

authors： Dejanovic B,Lal D,Catarino CB,Arjune S,Belaidi AA,Trucks H,Vollmar C,Surges R,Kunz WS,Motameny S,Altmüller J,Köhler A,Neubauer BA,Epicure Consortium,Nürnberg P,Noachtar S,Schwarz G,Sander T

更新日期：2014-07-01 00:00:00

abstract：:Neuroprotective functions of erythropoietin (Epo) are thought to involve a heteroreceptor composed of both Epo receptor (Epo-R) and common beta chain (betac). Here, we measured the response of hippocampal Epo system components (Epo, Epo-R and betac) during neurodegenerative processes following pilocarpine-induced stat...

journal_title：Neurobiology of disease

authors： Nadam J,Navarro F,Sanchez P,Moulin C,Georges B,Laglaine A,Pequignot JM,Morales A,Ryvlin P,Bezin L

更新日期：2007-02-01 00:00:00

abstract：:Parkinson's disease (PD) is a neurodegenerative disorder caused by loss of dopaminergic neurons. Although many reports have suggested that genetic factors are implicated in the pathogenesis of PD, molecular mechanisms underlying selective dopaminergic neuronal degeneration remain unknown. DJ-1 is a causative gene for ...

journal_title：Neurobiology of disease

authors： Usami Y,Hatano T,Imai S,Kubo S,Sato S,Saiki S,Fujioka Y,Ohba Y,Sato F,Funayama M,Eguchi H,Shiba K,Ariga H,Shen J,Hattori N

更新日期：2011-09-01 00:00:00

abstract：:Mutations in the leucine-rich repeat kinase 2 (LRRK2) gene cause late-onset, autosomal dominant Parkinson's disease (PD). The clinical and neurochemical features of LRRK2-linked PD are similar to idiopathic disease although neuropathology is somewhat heterogeneous. Dominant mutations in LRRK2 precipitate neurodegenera...

journal_title：Neurobiology of disease

authors： Tsika E,Kannan M,Foo CS,Dikeman D,Glauser L,Gellhaar S,Galter D,Knott GW,Dawson TM,Dawson VL,Moore DJ

更新日期：2014-11-01 00:00:00

abstract：:Huntington's disease (HD) is a late-onset neurodegenerative disease for which the mutation is CAG/polyglutamine repeat expansion. The R6 mouse lines expressing the HD mutation develop a movement disorder that is preceded by the formation of neuronal polyglutamine aggregates. The phenotype is likely caused by a widespr...

journal_title：Neurobiology of disease

authors： Liévens JC,Woodman B,Mahal A,Spasic-Boscovic O,Samuel D,Kerkerian-Le Goff L,Bates GP

更新日期：2001-10-01 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：:Altered expression and distribution of neurotransmitter receptors, including metabotropic glutamate receptors (mGluRs), constitute key aspects in epileptogenesis, impaired hippocampal excitability and neuronal degeneration. mGluR1 mediates predominantly excitatory effects, whereas mGluR4 acts as inhibitory presynaptic...

journal_title：Neurobiology of disease

authors： Pitsch J,Schoch S,Gueler N,Flor PJ,van der Putten H,Becker AJ

更新日期：2007-06-01 00:00:00