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

abstract：:In multiple sclerosis (MS), post-mortem studies of human brain tissue as well as data from animal models have shown that apoptosis of neurons occurs to a significant extent during this disease. As neurodegeneration in MS correlates with permanent neurological deficits in patients, understanding the mechanisms would be...

journal_title：Neurobiology of disease

authors： Diem R,Taheri N,Dietz GP,Kuhnert A,Maier K,Sättler MB,Gadjanski I,Merkler D,Bähr M

更新日期：2005-11-01 00:00:00

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

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

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

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

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

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

abstract：:Proteolytic cleavage of the amyloid precursor protein (APP) by the two proteases α- and β-secretases controls the generation of the amyloid β peptide (Aβ), a key player in Alzheimer's disease pathogenesis. The α-secretase ADAM10 and the β-secretase BACE1 have opposite effects on Aβ generation and are assumed to compet...

journal_title：Neurobiology of disease

authors： Colombo A,Wang H,Kuhn PH,Page R,Kremmer E,Dempsey PJ,Crawford HC,Lichtenthaler SF

更新日期：2013-01-01 00:00:00

abstract：:Several neurodegenerative diseases like Huntington's, a polyglutamine (PolyQ) disease, are initiated by protein aggregation in neurons. Furthermore, these diseases are also associated with a multitude of responses in non-neuronal cells in the brain, in particular glial cells, like astrocytes. These non-neuronal respon...

journal_title：Neurobiology of disease

authors： Bason M,Meister-Broekema M,Alberts N,Dijkers P,Bergink S,Sibon OCM,Kampinga HH

更新日期：2019-04-01 00:00:00

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

authors： Wisniewski T,Ghiso J,Frangione B

更新日期：1997-01-01 00:00:00

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

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

abstract：:Early exposure to general anesthesia (GA) causes developmental neuroapoptosis in the mammalian brain and long-term cognitive impairment. Recent evidence suggests that GA also causes functional and morphological impairment of the immature neuronal mitochondria. Injured mitochondria could be a significant source of reac...

journal_title：Neurobiology of disease

authors： Boscolo A,Starr JA,Sanchez V,Lunardi N,DiGruccio MR,Ori C,Erisir A,Trimmer P,Bennett J,Jevtovic-Todorovic V

更新日期：2012-03-01 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：:Heterozygous in frame duplications of the PHOX2B gene, leading to polyalanine (polyAla) expansions ranging from +5 to +13 residues of a 20-alanine stretch, have been identified in the vast majority of patients affected with Congenital Central Hypoventilation Syndrome (CCHS), a rare neurocristopathy characterized by ab...

journal_title：Neurobiology of disease

authors： Di Zanni E,Bachetti T,Parodi S,Bocca P,Prigione I,Di Lascio S,Fornasari D,Ravazzolo R,Ceccherini I

更新日期：2012-01-01 00:00:00

abstract：:Precursor cells in the adult dentate gyrus are a heterogeneous population. Astrocytic cell types with radial glia-like morphology and low proliferative activity have been distinguished from highly dividing subtypes expressing early neuronal properties. Recent evidence indicates that physiological stimuli predominantly...

journal_title：Neurobiology of disease

authors： Kunze A,Grass S,Witte OW,Yamaguchi M,Kempermann G,Redecker C

更新日期：2006-02-01 00:00:00

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

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

abstract：:Deep brain stimulation for epilepsy has garnered attention from epileptologists due to its well-documented success in treating movement disorders and the low morbidity associated with the implantation of electrodes. Given the large proportion of patients who fail medical therapy and are not candidates for surgical ame...

journal_title：Neurobiology of disease

authors： Lega BC,Halpern CH,Jaggi JL,Baltuch GH

更新日期：2010-06-01 00:00:00

abstract：:Schizophrenia is a complex neuropsychiatric disorder that involves disturbances in neural circuitry and synaptic function. The exquisite network architecture and capacity for discreet post-synaptic remodeling of neurons requires coordination by an elaborate intracellular network of molecular signal transduction system...

journal_title：Neurobiology of disease

authors： Beveridge NJ,Cairns MJ

更新日期：2012-05-01 00:00:00

abstract：:Hereditary sensory neuropathy type 1 (HSN-1) is a peripheral neuropathy most frequently caused by mutations in the SPTLC1 or SPTLC2 genes, which code for two subunits of the enzyme serine palmitoyltransferase (SPT). SPT catalyzes the first step of de novo sphingolipid synthesis. Mutations in SPT result in a change in ...

journal_title：Neurobiology of disease

authors： Wilson ER,Kugathasan U,Abramov AY,Clark AJ,Bennett DLH,Reilly MM,Greensmith L,Kalmar B

更新日期：2018-09-01 00:00:00

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

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

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

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

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

authors： Brera B,Serrano A,de Ceballos ML

更新日期：2000-08-01 00:00:00

abstract：:Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) is a recessively inherited disease characterized by early onset dementia associated with bone cysts. Our group has recently established the molecular background of PLOSL by identifying mutations in DAP12 and TREM2 genes. To understand...

journal_title：Neurobiology of disease

authors： Kiialainen A,Hovanes K,Paloneva J,Kopra O,Peltonen L

更新日期：2005-03-01 00:00:00

abstract：:Ischemic stroke remains a leading cause of disability worldwide. Surviving patients often suffer permanent neurological impairments, and spontaneous recovery rarely occurs. However, observations that early-life brain injuries, including strokes, elicit less severe long-term functional impairments, compared to adults, ...

journal_title：Neurobiology of disease

authors： Teo L,Boghdadi AG,de Souza M,Bourne JA

更新日期：2018-03-01 00:00:00

abstract：:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that results in motor, cognitive and psychiatric abnormalities. Dysfunction in neuronal processing between the cortex and the basal ganglia is fundamental to the onset and progression of the HD phenotype. The corticosubthalamic hyperdirect p...

journal_title：Neurobiology of disease

authors： Callahan JW,Abercrombie ED

更新日期：2015-06-01 00:00:00

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

authors： Orr HT

更新日期：2000-06-01 00:00:00

abstract：OBJECTIVE:In Parkinson's disease, chronic striatal dopamine depletion results in over-activity and under-activity of the indirect and direct striatal output pathways respectively. In this study, we investigated changes in the function of glutamatergic cortico-striatal synapses that contribute to abnormalities in striat...

journal_title：Neurobiology of disease

authors： Warre R,Thiele S,Talwar S,Kamal M,Johnston TH,Wang S,Lam D,Lo C,Khademullah CS,Perera G,Reyes G,Sun XS,Brotchie JM,Nash JE

更新日期：2011-03-01 00:00:00

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

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

abstract：:Neurodegenerative diseases encompass a large group of neurological disorders. Clinical symptoms can include memory loss, cognitive impairment, loss of movement or loss of control of movement, and loss of sensation. Symptoms are typically adult onset (although severe cases can occur in adolescents) and are reflective o...

journal_title：Neurobiology of disease

authors： Ambegaokar SS,Roy B,Jackson GR

更新日期：2010-10-01 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：:Multiple system atrophy is a progressive, neurodegenerative disease characterized by parkinsonism, ataxia, autonomic dysfunction, and accumulation of α-synuclein in oligodendrocytes. To understand how α-synuclein aggregates impact oligodendroglial homeostasis, we investigated an oligodendroglial cell model of α-synucl...

journal_title：Neurobiology of disease

authors： Kragh CL,Gysbers AM,Rockenstein E,Murphy K,Halliday GM,Masliah E,Jensen PH

更新日期：2014-03-01 00:00:00

abstract：:Synaptic neurodegeneration is thought to be an early event initiated by soluble β-amyloid (Aβ) aggregates that closely correlates with cognitive decline in Alzheimer disease (AD). Apolipoprotein ε4 (APOE4) is the most common genetic risk factor for both familial AD (FAD) and sporadic AD; it accelerates Aβ aggregation ...

journal_title：Neurobiology of disease

authors： Ma QL,Teng E,Zuo X,Jones M,Teter B,Zhao EY,Zhu C,Bilousova T,Gylys KH,Apostolova LG,LaDu MJ,Hossain MA,Frautschy SA,Cole GM

更新日期：2018-06-01 00:00:00

abstract：:While much of the research on neurodegenerative diseases has focused on neurons, non-neuronal cells are also affected. The extent to which glia and other non-neuronal cells are causally involved in disease pathogenesis versus more passively responding to disease is an area of active research. This is complicated by th...

journal_title：Neurobiology of disease

authors： Gleichman AJ,Carmichael ST

更新日期：2020-08-01 00:00:00