听力与言语-语言病理学

行为科学

医学伦理学

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  • Normal cognitive behavior in two distinct congenic lines of transgenic mice hyperexpressing mutant APP SWE.

    abstract::Amyloid deposition appears to be an early and crucial event in Alzheimer's disease (AD). To generate animal models of AD, mice expressing full-length amyloid precursor protein (APP), with mutations linked to FAD, have been created. These animals exhibit abnormalities characteristic of AD, including deposits of beta-am...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/s0969-9961(02)00012-8

    authors: Savonenko AV,Xu GM,Price DL,Borchelt DR,Markowska AL

    更新日期:2003-04-01 00:00:00

  • Complement-receptor-3 and scavenger-receptor-AI/II mediated myelin phagocytosis in microglia and macrophages.

    abstract::Microglia and macrophages express the alpha(M)/beta(2) integrin complement-receptor-3 (CR3/MAC-1; CD11b/CD18) and scavenger-receptor-AI/II (SRAI/II). Both can mediate myelin phagocytosis. We document that CR3/MAC-1 mediated myelin phagocytosis in microglia is modulated by complement and anti-CR3/MAC-1 mAbs. Complement...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1016/s0969-9961(02)00008-6

    authors: Reichert F,Rotshenker S

    更新日期:2003-02-01 00:00:00

  • Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression.

    abstract::In a transgenic mouse model of the neurodegenerative disorder Huntington's disease (HD), age-dependent neurologic defects are accompanied by progressive alterations in glucose tolerance that culminate in the development of diabetes mellitus and insulin deficiency. Pancreatic islets from HD transgenic mice express redu...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2002.0562

    authors: Andreassen OA,Dedeoglu A,Stanojevic V,Hughes DB,Browne SE,Leech CA,Ferrante RJ,Habener JF,Beal MF,Thomas MK

    更新日期:2002-12-01 00:00:00

  • Adenovirus-mediated Bcl-X(L) expression using a neuron-specific synapsin-1 promoter protects against disseminated neuronal injury and brain infarction following focal cerebral ischemia in mice.

    abstract::The effects of an adenovirus-mediated Bcl-X(L) expression, driven by a neuron-specific human synapsin-1 promoter, on the degree of injury, were examined after transient focal ischemia in mice. Therefore, injections of vehicle, of an adenoviral E1-deleted control vector (Ad-dE1), or a Bcl-X(L) vector (Ad-Syn-Bcl-X(L)) ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2002.0552

    authors: Kilic E,Hermann DM,Kügler S,Kilic U,Holzmüller H,Schmeer C,Bähr M

    更新日期:2002-11-01 00:00:00

  • Cerebrospinal fluid tissue transglutaminase as a biochemical marker for Alzheimer's disease.

    abstract::Tissue transglutaminase (tTG) is an indicator of acute cell death in vitro. An increase in tTG protein level is found in postmortem Alzheimer's disease (AD) brains as well as in Huntington's disease. No study revealed tTG in vivo so far. We investigated the concentrations of tTG in the cerebrospinal fluid (CSF) obtain...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2002.0535

    authors: Bonelli RM,Aschoff A,Niederwieser G,Heuberger C,Jirikowski G

    更新日期:2002-10-01 00:00:00

  • BDNF regulates the expression of fragile X mental retardation protein mRNA in the hippocampus.

    abstract::Both fragile X mental retardation protein (FMRP) and brain-derived neurotrophic factor (BDNF) are implicated in the maturation of neurons and in the higher cognitive functions. We have investigated whether FMRP and BDNF are reciprocally regulated in neurons. Exposure of cultured hippocampal neurons to BDNF, but not to...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2002.0544

    authors: Castrén M,Lampinen KE,Miettinen R,Koponen E,Sipola I,Bakker CE,Oostra BA,Castrén E

    更新日期:2002-10-01 00:00:00

  • Oxidative stress increases expression and activity of BACE in NT2 neurons.

    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

    pub_type: 杂志文章

    doi:10.1006/nbdi.2002.0515

    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

  • Ethanol-induced death of postnatal hippocampal neurons.

    abstract::Fetal alcohol exposure causes severe neuropsychiatric problems, but mechanisms of the ethanol-associated changes in central nervous system development are unclear. In vivo, ethanol's interaction with N-methyl-D-aspartate (NMDA) and gamma-aminobutyric acid type A (GABA(A)) receptors may cause increased apoptosis in the...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2002.0523

    authors: Moulder KL,Fu T,Melbostad H,Cormier RJ,Isenberg KE,Zorumski CF,Mennerick S

    更新日期:2002-08-01 00:00:00

  • Environmental risk factors and Parkinson's disease: selective degeneration of nigral dopaminergic neurons caused by the herbicide paraquat.

    abstract::Environmental toxicants and, in particular, pesticides have been implicated as risk factors in Parkinson's disease (PD). The purpose of this study was to determine if selective nigrostriatal degeneration could be reproduced by systemic exposure of mice to the widely used herbicide paraquat. Repeated intraperitoneal pa...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2002.0507

    authors: McCormack AL,Thiruchelvam M,Manning-Bog AB,Thiffault C,Langston JW,Cory-Slechta DA,Di Monte DA

    更新日期:2002-07-01 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

  • Destabilization of neuronal calcium homeostasis by factors secreted from choroid plexus macrophage cultures in response to feline immunodeficiency virus.

    abstract::The choroid plexus contains a major reservoir of macrophages poised for efficient delivery of virus and neurotoxins to the brain after infection by lentiviruses such as human or feline immunodeficiency virus (FIV). However, their contribution to neurotoxicity is poorly understood. Medium from FIV-infected, choroid ple...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2001.0459

    authors: Bragg DC,Boles JC,Meeker RB

    更新日期:2002-03-01 00:00:00

  • Insights into Abeta and presenilin from a canine model of human brain aging.

    abstract::In this review, we describe insights into beta-amyloid (Abeta) production using aged dogs as a model of human brain aging. The advantage of using dogs is that they naturally accumulate Abeta neuropathology with age. In parallel, dogs also develop age-associated learning and memory impairments. Thus, dogs can complemen...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1006/nbdi.2002.0476

    authors: Head E,Torp R

    更新日期:2002-02-01 00:00:00

  • Fetal hippocampal CA3 cell grafts transplanted to lesioned CA3 region of the adult hippocampus exhibit long-term survival in a rat model of temporal lobe epilepsy.

    abstract::Intracerebroventricular administration of kainic acid in the adult rat, a widely used model for studying human temporal lobe epilepsy, results in widespread degeneration of CA3-pyramidal neurons. Transplantation of specific fetal hippocampal CA3 cell grafts into the lesioned CA3-region at a prolonged post lesion delay...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2001.0440

    authors: Zaman V,Shetty AK

    更新日期:2001-12-01 00:00:00

  • Effects of exercise on gene-expression profile in the rat hippocampus.

    abstract::Exercise has beneficial effects on brain function, including the promotion of plasticity and the enhancement of learning and memory performance. Previously we found that exercise increases the expression of certain neurotrophic factors including brain derived neurotrophic factor in the rat hippocampus. To further expl...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2001.0427

    authors: Tong L,Shen H,Perreau VM,Balazs R,Cotman CW

    更新日期:2001-12-01 00:00:00

  • Impaired glutamate uptake in the R6 Huntington's disease transgenic mice.

    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

    pub_type: 杂志文章

    doi:10.1006/nbdi.2001.0430

    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

  • Insulin-like growth factor-I in muscle metabolism and myotherapies.

    abstract::The critical anabolic and trophic role of signaling by insulin-like growth factors (IGF) I and II via the type-I IGF receptor (IGF-IR) is reviewed throughout the life of skeletal myocytes. The proliferative effects of IGF-IR stimulation, both during embryogenesis and during satellite cell proliferation following dener...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1006/nbdi.2001.0416

    authors: Singleton JR,Feldman EL

    更新日期:2001-08-01 00:00:00

  • Adenovirus-mediated GDNF and CNTF pretreatment protects against striatal injury following transient middle cerebral artery occlusion in mice.

    abstract::During the last few years, adenoviral gene transfer techniques have achieved increasing interest in the treatment of neurodegenerative diseases. However, gene therapy requires that delivered genes are translated into proteins. This may pose a problem in focal ischemia where protein synthesis is compromized. The presen...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2001.0399

    authors: Hermann DM,Kilic E,Kügler S,Isenmann S,Bähr M

    更新日期:2001-08-01 00:00:00

  • Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA.

    abstract::Huntington's disease (HD) and Dentatorubral and pallidoluysian atrophy (DRPLA) are autosomal dominant, neurodegenerative disorders caused by the expansion of polyglutamine tracts in their respective proteins, huntingtin and atrophin-1. We have previously generated mouse models of these disorders, using transgenes expr...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2001.0385

    authors: Schilling G,Jinnah HA,Gonzales V,Coonfield ML,Kim Y,Wood JD,Price DL,Li XJ,Jenkins N,Copeland N,Moran T,Ross CA,Borchelt DR

    更新日期:2001-06-01 00:00:00

  • Lack of nigral pathology in transgenic mice expressing human alpha-synuclein driven by the tyrosine hydroxylase promoter.

    abstract::alpha-Synuclein has been identified as a major component of Lewy body inclusions, which are one of the pathologic hallmarks of idiopathic Parkinson's disease. Mutations in alpha-synuclein have been found to be responsible for rare familial cases of Parkinsonism. To test whether overexpression of human alpha-synuclein ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2001.0392

    authors: Matsuoka Y,Vila M,Lincoln S,McCormack A,Picciano M,LaFrancois J,Yu X,Dickson D,Langston WJ,McGowan E,Farrer M,Hardy J,Duff K,Przedborski S,Di Monte DA

    更新日期:2001-06-01 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

  • Seasonal affective disorder and serotonin-related polymorphisms.

    abstract::Disturbances in central serotonergic systems have been hypothesized to be involved in seasonal affective disorder (SAD). Association between SAD and the shorter allele of the serotonin transporter promoter repeat length polymorphism (5-HTTLPR) has been reported in an American sample. We have genotyped 82 SAD patients ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2000.0373

    authors: Johansson C,Smedh C,Partonen T,Pekkarinen P,Paunio T,Ekholm J,Peltonen L,Lichtermann D,Palmgren J,Adolfsson R,Schalling M

    更新日期:2001-04-01 00:00:00

  • Perinatal human hypoxia-ischemia vulnerability correlates with brain calcification.

    abstract::Deregulation of intracellular calcium homeostasis is widely considered as one of the underlying pathophysiological mechanisms of hypoxic-ischemic brain injury. Whether this alteration can result in cerebral calcification was investigated in basal ganglia, cerebral cortex, and hippocampus of human premature and term ne...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2000.0332

    authors: Rodríguez MJ,Ursu G,Bernal F,Cusí V,Mahy N

    更新日期:2001-02-01 00:00:00

  • Proapoptotic effects of tau cleavage product generated by caspase-3.

    abstract::Using an in vitro translation assay to screen a human brain cDNA library, we isolated the microtubule-associated protein Tau and determined it to be a caspase-3 substrate whose C-terminal cleavage occurred during neuronal apoptosis. DeltaTau, the 50-kDa cleavage product, was detected by Western blot in apoptotic corti...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2000.0335

    authors: Chung CW,Song YH,Kim IK,Yoon WJ,Ryu BR,Jo DG,Woo HN,Kwon YK,Kim HH,Gwag BJ,Mook-Jung IH,Jung YK

    更新日期:2001-02-01 00:00:00

  • p53 is abnormally elevated and active in the CNS of patients with amyotrophic lateral sclerosis.

    abstract::Motor neuron degeneration in amyotrophic lateral sclerosis (ALS) is a form of apoptosis, but the mechanisms for this neuronal cell death are not known. We evaluated whether motor neuron degeneration in ALS is associated with changes in the levels and function of the apoptosis regulating protein p53. The protein levels...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2000.0314

    authors: Martin LJ

    更新日期:2000-12-01 00:00:00

  • Differential screening of mutated SOD1 transgenic mice reveals early up-regulation of a fast axonal transport component in spinal cord motor neurons.

    abstract::In the present study we analyze the molecular mechanisms underlying motor neuron degeneration in familial amyotrophic lateral sclerosis (FALS). For this, we used a transgenic mouse model expressing the Cu/Zn superoxide dismutase (SOD1) gene with a Gly(86) to Arg (G86R) mutation equivalent to that found in a subset of ...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2000.0292

    authors: Dupuis L,de Tapia M,René F,Lutz-Bucher B,Gordon JW,Mercken L,Pradier L,Loeffler JP

    更新日期:2000-08-01 00:00:00

  • beta-amyloid peptides are cytotoxic to astrocytes in culture: a role for oxidative stress.

    abstract::beta-Amyloid is cytotoxic to neurons in culture by increasing hydrogen peroxide and altering calcium homeostasis. We have evaluated the cytotoxicty of beta-amyloid peptides (betaA(25-35) and betaA(1-40)) and generation of hydrogen peroxide on cortical cultured astrocytes. Twenty-four hours after a single addition of e...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.2000.0313

    authors: Brera B,Serrano A,de Ceballos ML

    更新日期:2000-08-01 00:00:00

  • The ins and outs of a polyglutamine neurodegenerative disease: spinocerebellar ataxia type 1 (SCA1).

    abstract::Polyglutamine neurodegenerative disorders are characterized by the expansion of a glutamine tract within the mutant disease-causing protein. Expression of the mutant protein induces a progressive loss of neuronal function and the subsequent neurodegeneration of a set of neurons characteristic to each disease. Spinocer...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1006/nbdi.2000.0305

    authors: Orr HT

    更新日期:2000-06-01 00:00:00

  • Oxidative insults are associated with apolipoprotein E genotype in Alzheimer's disease brain.

    abstract::The epsilon4 allele of the apolipoprotein E gene (APOE) is associated with sporadic and familial late-onset Alzheimer's disease (AD). Oxidative stress is believed to play an important role in neuronal dysfunction and cell death in AD. We now provide evidence that in the hippocampus of AD, the level of thiobarbituric a...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1999.0273

    authors: Ramassamy C,Averill D,Beffert U,Theroux L,Lussier-Cacan S,Cohn JS,Christen Y,Schoofs A,Davignon J,Poirier J

    更新日期:2000-02-01 00:00:00

  • Experimental models of amyotrophic lateral sclerosis.

    abstract::Amyotrophic lateral sclerosis (ALS) is a chronic neurodegenerative disease characterized by the progressive loss of motor neurons, leading to profound weakness and eventual death of affected individuals. For the vast majority of patients with ALS, the etiology of the disorder is unknown, and although multiple clinical...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1006/nbdi.1999.0266

    authors: Elliott JL

    更新日期:1999-10-01 00:00:00

  • Neuronal apoptosis induced by beta-amyloid is mediated by caspase-8.

    abstract::The Alzheimer disease-associated beta-amyloid peptide has been shown to induce apoptotic neuronal death. In the present study, we test the hypothesis that the apoptotic pathway activated by beta-amyloid is similar to the pathway activated by the Fas/TNFR family of death receptors, which requires caspase-8 activity and...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1999.0268

    authors: Ivins KJ,Thornton PL,Rohn TT,Cotman CW

    更新日期:1999-10-01 00:00:00

  • The role of copper in neurodegenerative disease.

    abstract::Copper is an essential trace metal which plays a fundamental role in the biochemistry of the human nervous system. Menkes disease and Wilson disease are inherited disorders of copper metabolism and the dramatic neurodegenerative phenotypes of these two diseases underscore the essential nature of copper in nervous syst...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1006/nbdi.1999.0250

    authors: Waggoner DJ,Bartnikas TB,Gitlin JD

    更新日期:1999-08-01 00:00:00

  • Involvement of oxidative stress on the impairment of energy metabolism induced by A beta peptides on PC12 cells: protection by antioxidants.

    abstract::Alzheimer's disease is widely held to be associated with oxidative stress due, in part, to the membrane action of amyloid beta-peptide (A beta) aggregates. In this study, the involvement of oxidative stress on A beta-induced energy metabolism dysfunction was evaluated on PC12 cells. It was shown that A beta peptides (...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1999.0241

    authors: Pereira C,Santos MS,Oliveira C

    更新日期:1999-06-01 00:00:00

  • Upregulation of COX-2 and CGRP expression in resident cells of the Borna disease virus-infected brain is dependent upon inflammation.

    abstract::Infection of immunocompetent adult rats with Borna disease virus (BDV) causes severe encephalitis and neural dysfunction. The expression of COX-2 and CGRP, genes previously shown to be implicated in CNS disease and peripheral inflammation, was dramatically upregulated in the cortical neurons of acutely BDV-infected ra...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1998.0225

    authors: Röhrenbeck AM,Bette M,Hooper DC,Nyberg F,Eiden LE,Dietzschold B,Weihe E

    更新日期:1999-02-01 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

  • Synaptic plasticity in the CA1 area of the hippocampus of scrapie-infected mice.

    abstract::Using conventional in vitro extracellular field potential recordings we have investigated both short- and long-term synaptic plasticity in the hippocampal CA1 area of mice infected with ME7 scrapie. In agreement with earlier studies, no changes were seen in the properties of the Schäffer collateralevoked field excitat...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1998.0194

    authors: Johnston AR,Fraser JR,Jeffrey M,MacLeod N

    更新日期:1998-09-01 00:00:00

  • A prion protein fragment primes type 1 astrocytes to proliferation signals from microglia.

    abstract::Giliosis is a hallmark of prion disease. A neurotoxic prion peptide (PrP106-126) induces astrocyte proliferation in the presence of microglia. This peptide also directly enhances microglial proliferation in culture. We have investigated this further to understand the method by which factors released by microglia and P...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1998.0169

    authors: Brown DR,Schmidt B,Kretzschmar HA

    更新日期:1998-04-01 00:00:00

  • Cellular distribution of NMDA glutamate receptor subunit mRNAs in the human cerebellum.

    abstract::We have used a quantitative in situ hybridization method with human ribonucleotide probes to examine the regional and cellular distribution of N-methyl-D-aspartate receptor (NMDAR) subunit mRNAs in the human cerebellum. Purkinje cells showed very dense labeling for NMDAR1 mRNA, dense labeling for NMDAR2A mRNA, and mod...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1997.0136

    authors: Scherzer CR,Landwehrmeyer GB,Kerner JA,Standaert DG,Hollingsworth ZR,Daggett LP,Veliçelebi G,Penney JB Jr,Young AB

    更新日期:1997-01-01 00:00:00

  • Frataxin shows developmentally regulated tissue-specific expression in the mouse embryo.

    abstract::Friedreich ataxia (FRDA) is an autosomal recessive degenerative disease caused either by an intronic GAA triplet repeat expansion that suppresses the expression of the frataxin gene on chromosome 9q13, or, rarely, by point mutations in the frataxin gene. We investigated the expression of the mouse frataxin homologue d...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1997.0139

    authors: Jiralerspong S,Liu Y,Montermini L,Stifani S,Pandolfo M

    更新日期:1997-01-01 00:00:00

  • Identification of candidate proteins binding to prion protein.

    abstract::Prion diseases are disorders of protein conformation that produce neurodegeneration in humans and animals. Studies of transgenic (Tg) mice indicate that a factor designated protein X is involved in the conversion of the normal cellular prion protein (PrPC) into the scrapie isoform (PrPSc); protein X appears to interac...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章

    doi:10.1006/nbdi.1997.0130

    authors: Yehiely F,Bamborough P,Da Costa M,Perry BJ,Thinakaran G,Cohen FE,Carlson GA,Prusiner SB

    更新日期:1997-01-01 00:00:00

  • Biology of A beta amyloid in Alzheimer's disease.

    abstract::The genetic associations with the pathological features of AD are diverse: A rapidly growing number of mutations in presenilin 1 and 2 on chromosomes 14 and 1, respectively, are found in many early-onset FAD patients (Lendon et al., 1997). In addition, beta PP mutations are found in a small percentage of early-onset F...

    journal_title:Neurobiology of disease

    pub_type: 杂志文章,评审

    doi:10.1006/nbdi.1997.0147

    authors: Wisniewski T,Ghiso J,Frangione B

    更新日期:1997-01-01 00:00:00

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