• The predisposition to hepatotoxicity results directly from the keratin mutation since nontransgenic or transgenic mice that express normal human K18 are more resistant. (jci.org)
  • Tg2576 mice overexpress a mutant form of human amyloid precursor protein with the Swedish mutation (APP Sw ), resulting in high β-amyloid (Aβ) levels in the brain. (jneurosci.org)
  • A founder transgenic mouse harbored two different integration patterns ofa transgene at the same locus, each of which gave rise to a similar autosomal recessive mutation. (researchwithrutgers.com)
  • Figure 1: Conditional mouse model of type A NPM1c mutation. (nature.com)
  • Mastrianni JA , Capellari S , Telling GC , Han D , Bosque P , Prusiner SB , Inherited prion disease caused by the V210I mutation: transmission to transgenic mice. (cdc.gov)
  • Chiesa R , Drisaldi B , Quaglio E , Migheli A , Piccardo P , Ghetti B , Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation. (cdc.gov)
  • Castilla J , Gutierrez-Adan A , Brun A , Pintado B , Salguero FJ , Parra B , Transgenic mice expressing bovine PrP with a four extra repeat octapeptide insert mutation show a spontaneous, non-transmissible, neurodegenerative disease and an expedited course of BSE infection. (cdc.gov)
  • The mutation leads to the abnormal expansion of the production of the polyglutamine tract (polyQ) resulting in the form of an unstable Huntingtin protein commonly referred to as mutant Huntingtin. (benthamscience.com)
  • Mutant Huntingtin is the cause of the complex neurological metabolic alteration of Huntington's disease, resulting in both the loss of all the functions of normal Huntingtin and the genesis of abnormal interactions due to the presence of this mutation. (benthamscience.com)
  • 2 The polyglutamine expansion mutation causes disease by conferring a novel deleterious function on the mutant protein and the severity correlates with increasing CAG repeat number and expression levels in transgenic mice and in cell culture models. (bmj.com)
  • While the experiments in the current paper were in progress, Leavitt et al 7 provided in vivo evidence suggesting that wild type huntingtin can protect against the gain of function mutation caused by the expanded polyglutamine tract in mutant huntingtin, using a YAC transgenic mouse model. (bmj.com)
  • The mutant huntingtin protein is ubiquitously expressed, but only certain brain regions are affected. (lu.se)
  • Further understanding of mechanisms rendering neurons sensitive to mutant huntingtin may reveal novel targets for therapeutic interventions. (lu.se)
  • OBJECTIVES Recent data suggest that wild type huntingtin can protect against apoptosis in the testis of mice expressing full length huntingtin transgenes with expanded CAG repeats. (bmj.com)
  • It is not clear if this protective effect was confined to particular cell types, or if wild type huntingtin exerted its protective effect in this model by simply reducing the formation of toxic proteolytic fragments from mutant huntingtin. (bmj.com)
  • RESULTS Full length wild type huntingtin significantly reduced cell death resulting from the mutant HD exon 1 fragments containing 72 CAG repeats in both cell lines. (bmj.com)
  • CONCLUSIONS Our results suggest that wild type huntingtin can significantly reduce the cellular toxicity of mutant HD exon 1 fragments in both neuronal and non-neuronal cell lines. (bmj.com)
  • This suggests that wild type huntingtin can be protective in different cell types and that it can act against the toxicity caused by a mutant huntingtin fragment as well as against a full length transgene. (bmj.com)
  • N-terminal fragments of mutant huntingtin accumulate and form inclusions in the cell nucleus in the brains of patients with HD, as well as in various animal and cell models of HD. (medscape.com)
  • [ 7 ] More recent data from striatal neuronal cultures transfected with mutant huntingtin and transgenic mice carrying the spinocerebellar ataxia-1 (SCA-1) gene (another CAG repeat disorder) suggest that NIIs may not be necessary or sufficient to cause neuronal cell death, but translocation into the nucleus is sufficient to cause neuronal cell death. (medscape.com)
  • Quantification of Total and Mutant Huntingtin Protein Levels in Biospecimens Using a Novel alphaLISA Assay. (lu.se)
  • Effects of Deletion of Mutant Huntingtin in Steroidogenic Factor 1 Neurons on the Psychiatric and Metabolic Phenotype in the BACHD Mouse Model of Huntington Disease. (lu.se)
  • She was the first author on a paper published in 1990 in Science, entitled "Spontaneous neurodegeneration in transgenic mice with mutant prion protein", describing the creation of a transgenic mouse modeling a neurodegenerative disease. (wikipedia.org)
  • Spontaneous neurodegeneration in transgenic mice with mutant prion protein. (cdc.gov)
  • The c-Abl inhibitor IkT-148009 suppresses neurodegeneration in mouse models of heritable and sporadic Parkinson's disease. (neurotree.org)
  • Another round of cat-and-mouse between scientists and the surreptitious cause of Alzheimer's pathogenesis shows intracellular Aβ lesions lead to age-dependent neurodegeneration and motor impairment in rodents. (alzforum.org)
  • Lastly, we recently found that the Ataxin-1 paralog (Ataxin-1 like) can suppress neurodegeneration in the SCA1 knock-in mice by displacing Ataxin-1 from its native complex facilitating its sequestration into inclusions. (bcm.edu)
  • To begin to understand how mutations in MATR3 may cause disease, here we provide initial characterization of transgenic (Tg) mice expressing human wild-type (WT) MATR3 (MATR3 WT ) and ALS-mutant F115C MATR3 (MATR3 F115C ) proteins under the control of the mouse prion promoter (MoPrP). (biomedcentral.com)
  • 20 months were not overtly distinguishable from non-transgenic (NT) littermates based on basic motor phenotype. (biomedcentral.com)
  • Mice of the mutant phenotype were of small stature but had normal levels of growth hormone. (researchwithrutgers.com)
  • We demonstrate that deletion of ERRγ from DAergic neurons in adult mice was sufficient to cause a levodopa-responsive PD-like phenotype with reductions in mitochondrial gene expression and number, that partial deficiency of ERRγ hastens synuclein-mediated toxicity, and that ERRγ overexpression reduces inclusion load and delays synuclein-mediated cell loss. (nature.com)
  • However, this is difficult to model in mice because loss of one TBK1 allele in mice does not cause an overt phenotype but a complete knockout of this gene causes embryonic lethality. (neurodegenerationresearch.eu)
  • We will inject the rAAV into several mouse strains including the normal wild type and mutant transgenic mice that develop ALS phenotype. (neurodegenerationresearch.eu)
  • Imbalance of the oxytocin-vasopressin system contributes to the neuropsychiatric phenotype in the BACHD mouse model of Huntington disease. (lu.se)
  • [ 1 ] A recent hypothesis to explain the migraine-stroke association, based on experimental data obtained in mice expressing familial hemiplegic migraine type 1 mutations, is that the cerebral hyperexcitability phenotype associated with migraine might sensitize brain tissue to ischemia. (medscape.com)
  • In addition, the increased expression of transthyretin and insulin-like growth factor-2 and the increased phosphorylation of Bad in hippocampal neurons were maintained in 12-month-old APP Sw mice when compared with age-matched controls. (jneurosci.org)
  • These results suggest that the slow progression and lack of full-fledged Alzheimer's disease pathology in the hippocampal neurons of APP Sw mice result from the genetic reprogramming of neural cells to cope with increased levels of Aβ. (jneurosci.org)
  • Thus the present study was designed to determine gene expression profiles in the hippocampus and cerebellum of 6-month-old APP Sw mice compared with age-matched controls and to identify potential mechanisms responsible for protecting neurons from Aβ toxicity. (jneurosci.org)
  • We found a significant reduction in the dopaminergic neurons in the substantia nigra and the dopamine content in the striatum in the high-expressing I93M Tg mice as compared with non-Tg mice at 20 weeks of age. (elsevierpure.com)
  • Although these changes were absent in the low-expressing I93M Tg mice, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) treatment profoundly reduced dopaminergic neurons in this line as compared with wild-type Tg or non-Tg mice. (elsevierpure.com)
  • Abnormal neuropathologies were also observed, such as silver staining-positive argyrophilic grains in the perikarya of degenerating dopaminergic neurons, in I93M Tg mice. (elsevierpure.com)
  • Altogether, these experiments suggest that ERRγ-deficient mice could provide a model for understanding the regulation of transcription in DAergic neurons and that amplifying ERRγ - mediated transcriptional programs should be considered as a strategy to promote DAergic maintenance in PD. (nature.com)
  • Matthias Staufenbiel of the Novartis Institutes for Biomedical Research in Basel, Switzerland, and colleagues created genetically modified mice that produce abundant levels of Aβ40 and/or Aβ42 within their neurons. (alzforum.org)
  • While the paper directly demonstrates that Aβ42 aggregates inside neurons cause neuronal death even in young mice, some other scientists express skepticism that the study settles the role of intraneuronal Aβ in AD. (alzforum.org)
  • In paper №2, I describe how mesDA neurons transplanted in the adult SN of a PD mouse model, extended axons across millimetres into the striatum, functionally reforming the nigrostriatal pathway. (lu.se)
  • The disrupted locus was cloned, and a genetic and molecular analysis showed that the insertional mutants were alielic to a spontaneous mutant, pygmy. (researchwithrutgers.com)
  • Jackson WS , Borkowski AW , Faas H , Steele AD , King OD , Watson N , Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice. (cdc.gov)
  • The directed expression of SV40 T antigen has led to the development of several important transgenic models with spontaneous epithelial tumor formation. (molvis.org)
  • As a result, ADAR1 Zα-domain mutant human cells and transgenic mice develop a spontaneous MDA5-dependent immune response. (biorxiv.org)
  • In contrast, T4K and T17M rhodopsins express at high levels, exhibit little or no misfolding and localize primarily to the OS in transgenic frog rods suggesting that ER stress may not play a major role in their pathology. (jneurosci.org)
  • Senescence-related impairment of autophagy induces toxic intraneuronal amyloid-β accumulation in a mouse model of amyloid pathology. (uclouvain.be)
  • Sustained APPsα overexpression in aged mice with already preexisting pathology and amyloidosis restored synaptic plasticity and partially rescued spine density deficits. (springer.com)
  • We will determine whether this approach can induce ALS phenotypes in the wild type mice and/or modulate the disease phenotypes and pathology in the mutant ALS transgenic mice. (neurodegenerationresearch.eu)
  • Despite this, amyloid plaques do not develop until 12 months of age, and there is no neuronal loss in mice as old as 16 months. (jneurosci.org)
  • These mice, therefore, may not be a good model of the complete pathologic process of AD but rather may be an excellent model for understanding how the brain can adapt to and survive high levels of Aβ. (jneurosci.org)
  • A transgenic mouse model was developed by introducing a human mutant UMOD (C148W) cDNA under control of the mouse umod promoter. (elsevierpure.com)
  • The mice should be a useful model for the growth hormone-resistant human dwarf syndromes and could lead to a greater understanding of the pathways involved in growth and development. (researchwithrutgers.com)
  • Using semi-quantitative reverse-transcription polymerase chain reaction (RT-PCR) and western blots experiments, we compared changes in ETS-1 and ETS-2 expression, their protein levels, and the regulation of some of their target gene expressions at different stages of the ocular tumoral progression in the transgenic mouse model, Tyrp-1-TAg, with those in normal eyes from control mice of the same age. (molvis.org)
  • We demonstrate that the R6/2 transgenic mouse model expressing a short fragment of mutant HTT displays hypothalamic neuropathology with discrete loss of the neuronal populations expressing orexin, MCH, CART, and orexin at 12 weeks of age. (lu.se)
  • The BACHD mouse model expressing full-length mutant HTT does not display any hypothalamic neuropathology at 2 months of age. (lu.se)
  • There was no effect of hypothalamic injections of QA on the neuronal populations expressing orexin, MCH, CART or oxytocin in neither HD mouse model. (lu.se)
  • Here, we used a gene therapy approach to directly overexpress APPsα in the brain using AAV-mediated gene transfer and explored its potential to rescue structural, electrophysiological and behavioral deficits in APP/PS1∆E9 AD model mice. (springer.com)
  • We use a range of animal model systems (chick, mouse, zebrafish) and combine in vivo and ex vivo approaches with state-of-the-art transcriptomic, imaging, and gain-and loss-of-function approaches. (sheffield.ac.uk)
  • This study investigated effects of the aqueous extract of its leaves (ATL) on cognitive and emotional deficits using an olfactory bulbectomized mouse (OBX) model. (hindawi.com)
  • We created a mouse model that expresses the glutamine-expanded Ataxin-1 under the control of endogenous locus (knock-in SCA1 mice) and found that this model recapitulates all features of SCA1. (bcm.edu)
  • This model predicts that most proteins that interact with wild-type Ataxin-1 should interact with the mutant protein and that modifications of Ataxin-1 that might alter its conformation are critical to pathogenesis. (bcm.edu)
  • However, the conventional transgenic technologies of gene overexpression and knockout are slow and expensive, thus becoming a bottleneck constraining new model development. (neurodegenerationresearch.eu)
  • If successful, this experiment will establish a new mouse model for ALS and a new method that is faster than the conventional gene knockout approach for construction of mouse models for ALS and for studying mutant gene interactions in vivo. (neurodegenerationresearch.eu)
  • [ 2 ] The main drawback of this theoretical construct is that it comes from a transgenic animal model of monogenic migraine. (medscape.com)
  • that is histopathological y very simi- T cel s, B cel s, natural kil er cel s, LMP1 of EBV can transform ro- lar to that caused by hepatitis B vi- macrophages, and dendritic cells, dent fibroblasts and is expressed rus (HBV) in humans, but it does so and this humanized mouse model in most of the human cancers as- through a different mechanism. (who.int)
  • In paper №1, I detail the neurodegenerative and behavioural outcomes in a mouse lesion model of PD, which can be used as a platform for the development of novel therapeutic strategies. (lu.se)
  • Here we report that activation of a humanized Npm1c knock-in allele in mouse hemopoietic stem cells causes Hox gene overexpression, enhanced self renewal and expanded myelopoiesis. (nature.com)
  • Direct overexpression of intracellular Aβ1-40 and Aβ1-42 in mice is extremely artificial,' wrote Virginia Lee of the University of Pennsylvania School of Medicine in Philadelphia in an e-mail to Alzforum. (alzforum.org)
  • Podocyte-specific overexpression of human angiotensin-converting enzyme 2 attenuates diabetic nephropathy in mice. (ohri.ca)
  • Podocyte Injury and Albuminuria in Mice with Podocyte-Specific Overexpression of the Ste20-Like Kinase, SLK. (ohri.ca)
  • We tested this hypothesis in cells, fruit flies, and mice and found that indeed chaperone overexpression mitigates SCA1 neuropathology and phenotypes. (bcm.edu)
  • In this study, transgenic and nontransgenic control mice were generated from C57B6/SJL N2 generation Tg2576 mice backcrossed to C57B6/SJL breeders. (jneurosci.org)
  • The expression of transthyretin, a protein shown to sequester Aβ and prevent amyloid fibril formation in vitro , and several genes in the insulin-signaling pathway, e.g., insulin-like growth factor-2, were increased selectively in the hippocampus of APP Sw mice. (jneurosci.org)
  • Embryonic stem (ES) cells are important vehicles for transfer of genes into mice. (cshlpress.com)
  • Inoculation with a high dose strains of LMP1 transgenic mice vide a powerful tool in mechanistic of EBV caused a B-cell lymphopro- were established that express LMP1 studies on the role of individual viral liferative disorder in these mice, under the control of the immunoglob- genes in cancer. (who.int)
  • Our results indicate that hepatocyte intermediate filament disruption renders mice highly susceptible to hepatotoxicity, and raises the possibility that K18 mutations may predispose to drug hepatotoxicity. (jci.org)
  • One third of mice developed delayed-onset AML, suggesting a requirement for cooperating mutations. (nature.com)
  • Induction of protective immunity by vaccination with wild-type apo superoxide dismutase 1 in mutant SOD1 transgenic mice. (ulaval.ca)
  • Vaccinations targeting extracellular superoxide dismutase 1 (SOD1) mutants are beneficial in mouse models of amyotrophic lateral sclerosis (ALS). (ulaval.ca)
  • The finding of a secretion pathway and toxicity for mutant superoxide dismutase 1 (SOD1) raised up the possibility of using immunization approaches to reduce or neutralize the burden of toxic SOD1 species in the nervous system. (nih.gov)
  • Targeted gene deletion mutants of amlE and amlR were constructed by use of the CRISPR/Cas9 technology. (frontiersin.org)
  • Transcription of the aml operon is significantly repressed in the wild type when growing on glucose and repression is absent in an Δ amlR deletion mutant. (frontiersin.org)
  • Ubiquitin C-terminal hydrolase L1 deletion ameliorates glomerular injury in mice with ACTN4-associated focal segmental glomerulosclerosis. (ohri.ca)
  • Keratin glycosylation decreased in all griseofulvin-fed mice, whereas keratin phosphorylation increased dramatically preferentially in mice expressing normal K18. (jci.org)
  • Transgenic animal models expressing rhodopsin glycosylation mutants also exhibit light exacerbated retinal degeneration (RD). In this study, we used transgenic Xenopus laevis to investigate the pathogenic mechanism connecting light exposure and RD in photoreceptors expressing T4K or T17M rhodopsin. (jneurosci.org)
  • TRACK-HD is a prospective observational study that reported 12-month longitudinal changes in 116 pre-manifest individuals carrying the mutant Huntington gene (preHD), 114 patients with early HD, and 115 age- and sex-matched controls. (medscape.com)
  • Although the previously reported mice develop Aβ deposits, they do not show signs of overt toxicity, and so speak to a possibility that intraneuronal Aβ aggregates are among the saboteurs in AD. (alzforum.org)
  • 1992. Preparation of an optimum mobile phase for the simultaneous determination of neurochemicals in mouse brain tissues by high-performance liquid chromatography with electrochemical detection. . (oregonstate.edu)
  • ETS-1 and ETS-2 mRNA and protein levels were much higher in the ocular tissues of Tyrp-1-TAg mice than in control ocular tissues from wild-type mice. (molvis.org)
  • We have studied the transgenic mouse strain, Tyrp-1-TAg, to try to gain insight into possible molecular mechanisms common to pigmented ocular neoplasms occurring spontaneously in the eyes of these mice and human choroidal melanoma. (molvis.org)
  • Uromodulin accumulation was observed in the thick ascending limb cells in the kidney of transgenic mice. (elsevierpure.com)
  • Relevant mouse models of human uveal melanoma still remain to be developed. (molvis.org)
  • Unlike cancer arising in the human population, tumors in genetically engineered mouse models arise in mice with well defined genetic backgrounds where genetic variability can be minimized. (molvis.org)
  • Even so, the novel approach to creating Aβ transgenic models can teach us about the basic biology of these proteins, said Nilsson. (alzforum.org)
  • Aydin D, Weyer SW, Muller UC (2012) Functions of the APP gene family in the nervous system: insights from mouse models. (springer.com)
  • To solve these problems, we propose a rAAV-intrathecal gene delivery approach for construction of transgenic mouse models for ALS. (neurodegenerationresearch.eu)
  • Transgenic mouse models pro- fection. (who.int)
  • We compared the effects of WT-apo to those of a G93A SOD1 vaccine in low-copy G93A SOD1 transgenic mice. (ulaval.ca)
  • On the other hand, the sera from G93A SOD1-vaccinated mice showed higher IFNγ or tumor necrosis factor and yielded a lower IgG1/IgG2c ratio than the sera from WT-apo-vaccinated mice. (ulaval.ca)
  • Here we tested a passive immunization approach based on intracerebroventricular infusion in G93A-SOD1 mice of monoclonal antibodies specific to misfolded forms of SOD1 (mSOD1). (nih.gov)
  • One antibody succeeded in reducing the level of mSOD1 by 23% in the spinal cord and in prolonging the lifespan of G93A-SOD1 mice in proportion to the duration of treatment. (nih.gov)
  • Interestingly, the variable Fab fragment of an anti-SOD1 antibody was sufficient to confer some protection in G93A-SOD1 mice. (nih.gov)
  • Genetic studies in mice and fruit flies have shed the most light on SCA1 pathogenesis. (bcm.edu)
  • However, this process is slow because of the low yield of double or triple transgenic mice and the multigenerational crosses that are necessary to bring different transgenic lines to the same genetic background. (neurodegenerationresearch.eu)
  • Unexpectedly, for all stably-transmitting lines examined, MATR3 transgenic mRNA expression was more robust in muscle, with minimal expression in spinal cord. (biomedcentral.com)
  • Transgenic mice had abundant microglial expression of signal transducers and activators of transcription 4, an activator of transcription of IFNγ, in the spinal cord implicating IFNγ in the pathogenesis. (ulaval.ca)
  • Moreover, the creatinine clearance was not different between wildtype and transgenic animals. (elsevierpure.com)
  • This new and unprecedented evidence for a link between SdhD dysfunction and p21(WAF1/Cip1) will open new avenues for the study of the mechanisms that cause tumors in Sdh mutants. (ed.ac.uk)
  • Concomitant activation of the insulin-like growth factor-1 receptor, Akt, and extracellular signal-regulated protein kinase 1 and 2 as well as increased phosphorylation of Bad also were unique to the hippocampus of APP Sw mice. (jneurosci.org)
  • The levels of transgenic mRNA in muscle did not differ between mice from our lead MATR3 F115C line and lead MATR3 WT line, but mice from the lead MATR3 F115C line had significantly higher levels of MATR3 protein in muscle over the lead MATR3 WT line. (biomedcentral.com)
  • Because of its misfolded nature, wild-type nonmetallated SOD1 protein (WT-apo) may have therapeutic application for vaccination of various SOD1 mutants. (ulaval.ca)
  • Glutamine-expanded Ataxin-1 causes disease by a gain-of-function mechanism given that mice lacking this protein do not reproduce SCA1 features. (bcm.edu)
  • Our analysis of SCA1 transgenic mice revealed that the mutant protein aggregates in the nuclei of cerebellar Purkinje cells and that these aggregates stain positively for ubiquitin, the proteasome, and some chaperones. (bcm.edu)
  • Nephropathy and Elevated BP in Mice with Podocyte-Specific NADPH Oxidase 5 Expression. (ohri.ca)
  • The higher sensitivity of ASXL1 mutant cells to the combination treatment was confirmed in vivo in ASXL1 Y588X transgenic mice. (haematologica.org)
  • For instance, mice are able to reconstitute most lymphomas in monkeys and humans woodchuck hepatitis virus induces major components of the human provides strong support for a direct hepatocellular carcinoma (HCC) haematolymphoid system including oncogenic role of EBV in vivo. (who.int)
  • The formation of extracellular plaques is common to all lines of transgenic mice overexpressing high levels of mutant APP. (jneurosci.org)
  • We also demonstrated upregulation of ETS-1 and ETS-2 target expressions in Tyrp-1-TAg mice when comparing with the same target expressions in control mice. (molvis.org)
  • For each construct, we established multiple independent lines of mice that stably transmitted the transgene. (biomedcentral.com)
  • In the present study, we wanted to examine whether transgenic HD mice display altered sensitivity to excitotoxicity in the hypothalamus. (lu.se)
  • The absence of Parkin does not promote dopamine or mitochondrial dysfunction in PolgA mitochondrial mutator mice. (neurotree.org)
  • Susceptibility to hepatotoxicity in transgenic mice that express a dominant-negative human keratin 18 mutant. (jci.org)
  • We recently described transgenic mice that express point-mutant human K18 (Ku, N.-O., S. Michie, R.G. Oshima, and M.B. Omary. (jci.org)
  • Using ADAR1 Zα-domain mutant human cells and knock-in mice, we demonstrate that abrogated binding of ADAR1 to Z-RNA leads to reduced A-to-I editing of dsRNA structures formed by pairing of inversely oriented SINEs. (biorxiv.org)
  • In this combined immunodeficiency (SCID) cancer is low in these species (as it chapter, some aspects of this issue mice, in which the human target is in humans), which renders cancer are discussed. (who.int)
  • Immunoblots of brain extracts from hemizygous 113L BoPrP-Tg037 +/− 113L BoPrP-Tg009 +/− mouse lines compared with those of cow brain extract and BoPrP-Tg110 mouse brain extract. (cdc.gov)
  • The authors used histochemical analysis to examine brain sections of mice that expressed either Aβ40 or Aβ42. (alzforum.org)
  • Along with the observation that migraine mutants had an elevated minimum cerebral blood flow threshold required for tissue survival and developed larger infarcts, these findings directly support the hypothesis that brain tissue in migraineurs is more susceptible to ischemic injury. (medscape.com)
  • We first assessed effects of hypothalamic injections of the excitotoxin quinolinic acid (QA) into wild-type (WT) mice. (lu.se)
  • In collaboration with Juan Botas (Baylor College of Medicine), we found that high levels of wild-type Ataxin-1 produce effects similar to mutant Ataxin-1 in Drosophila and mice. (bcm.edu)
  • Consequently, the onset of the disease was not observed in transgenic mice until 24 weeks of age. (elsevierpure.com)
  • Gene expression profiles in the hippocampus and cerebellum of 6-month-old APP Sw mice were compared with age-matched controls. (jneurosci.org)
  • Therefore, we were interested in early gene expression changes that might mediate neuroprotection in these mice. (jneurosci.org)
  • Our results revealed that each mutant tissue displayed different variations in their gene expression profiles affecting to different biological processes. (ed.ac.uk)
  • Szabo V, Kreienkamp HJ, Rosenberg T, Gal A. p.Gln200Glu, a putative constitutively active mutant of rod alpha-transducin (GNAT1) in autosomal dominant congenital stationary night blindness. (medlineplus.gov)
  • The combined transgenic exhibited intermediate levels of aggregation and pathologies, supporting the possibility that Aβ40 destabilizes the aggregation of Aβ42. (alzforum.org)
  • Here we show that mutant K18 expressing transgenic mice are highly susceptible to hepatotoxicity after acute administration of acetaminophen (400 mg/Kg) or chronic ingestion of griseofulvin (1.25% wt/wt of diet). (jci.org)
  • Figure 2: Hematopoietic changes and incidence of AML in Npm1 cA/+ mice. (nature.com)
  • In normal control adult mouse eyes, ETS-1 was mostly present in the nuclei of all neuroretinal layers whereas ETS-2 was mostly localized in the cytosol of the cell bodies of these layers with a smaller amount present in the nuclei. (molvis.org)