Search Results - (Author, Cooperation:A. Aguzzi)

2013-01-12

American Association for the Advancement of Science (AAAS)

0036-8075

1095-9203

Biology

Chemistry and Pharmacology

Computer Science

Medicine

Natural Sciences in General

Physics

Alzheimer Disease/immunology/physiopathology/therapy ; Animals ; Humans ; Mental Disorders/immunology/*physiopathology/therapy ; Microglia/cytology/immunology/*physiology ; Neurodegenerative Diseases/immunology/*physiopathology/therapy ; Neurogenic Inflammation/immunology/*physiopathology ; Neuroimmunomodulation/*physiology

2014-08-08

Nature Publishing Group (NPG)

0028-0836

1476-4687

Biology

Chemistry and Pharmacology

Medicine

Natural Sciences in General

Physics

Alzheimer Disease/*classification/epidemiology/*metabolism/therapy ; Amyloid beta-Peptides/*chemistry/*classification/genetics/metabolism ; Animals ; Antibody Specificity ; Arctic Regions ; Cattle ; Humans ; Mice ; *Models, Biological ; Prion Diseases/classification/metabolism/pathology/transmission ; Protein Conformation

2013-08-02

Nature Publishing Group (NPG)

0028-0836

1476-4687

Biology

Chemistry and Pharmacology

Medicine

Natural Sciences in General

Physics

Amino Acid Sequence ; Animals ; Antibodies/*immunology/*toxicity ; Antibodies, Monoclonal/immunology/toxicity ; Binding Sites, Antibody ; Calpain/metabolism ; Cerebellum ; Creutzfeldt-Jakob Syndrome/metabolism ; Cross-Linking Reagents ; Epitope Mapping ; Female ; Immunoglobulin Fab Fragments/immunology/toxicity ; In Vitro Techniques ; Ligands ; Male ; Membrane Glycoproteins/metabolism ; Mice ; Molecular Sequence Data ; NADPH Oxidase/metabolism ; Neurodegenerative Diseases/metabolism ; Oxidative Stress ; *Pliability ; PrPC Proteins/chemistry/genetics/immunology ; Prions/*chemistry/genetics/*immunology ; Reactive Oxygen Species/metabolism ; Sequence Deletion/genetics ; Single-Chain Antibodies/immunology/toxicity

2018-03-06

Rockefeller University Press

0022-1007

1540-9538

Medicine

Neuroscience

1600-0560

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Electronic Resource

1460-9568

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Replication-defective retroviruses expressing the t-neu oncogene, or a hybrid protein with the neu tyrosine kinase linked to the external region of the human epidermal growth factor receptor (egfr-neu), were used to establish lines of murine oligodendroglial precursor cells. Differentiation of the t-neu lines into myelin-associated glycoprotein (MAG)-positive oligodendrocytes was induced by dibutyryl cAMP, and the egfr-neu line showed limited differentiation in vitro upon withdrawal of epidermal growth factor. Cerebellar granule cell neurons expressed mitogens for the cell lines. Upon transplantation into demyelinated lesions, t-neu line cells engaged with the demyelinated axons whereas the egfr-neu line cells differentiated further and ensheathed the axons. These cell lines thus interact with neurons in vitro and in vivo and can be used as tools to define the molecules involved in different stages of neuron-glia interaction.

Electronic Resource

1365-2516

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Summary. Although the nature of the infectious agent causing prion diseases is still debated, several of its molecular characteristics have been clarified in remarkable detail. The transmissibility of bovine spongiform encephalopathy to humans dramatically highlights the need for research focused at interference with prion replication and spread, and at prevention of brain damage. Precondition to achieving these goals is a thorough understanding of prion biology, and in particular of its protein chemistry.

Electronic Resource

1749-6632

Blackwell Publishing Journal Backfiles 1879-2005

Natural Sciences in General

Electronic Resource

1750-3841

Blackwell Publishing Journal Backfiles 1879-2005

Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition

Process Engineering, Biotechnology, Nutrition Technology

: This study provides data on the total heme and non-heme iron contents in poultry (chicken, turkey), beef, veal, lamb, horse, ostrich, rabbit, and pork meat cuts. The effect of cooking on heme iron content was also studied. Total iron and heme iron contents markedly differed between muscles in poultry. Heme iron in red meats ranged from 72 to 87%. Heme iron in rabbit and pork was 56 and 62% of total iron. Heating decreased heme iron, the severity of the losses depended on cooking methods: in poultry, losses ranged from 22 to 43%; less severe impact was detected in pan-cooked meat, where the losses ranged from 1 to 24%.

Electronic Resource

1365-2559

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Electronic Resource

1365-2559

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Electronic Resource

0304-419X

Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Medicine

Electronic Resource

1749-6632

Blackwell Publishing Journal Backfiles 1879-2005

Natural Sciences in General

The amyloid precursor protein (βAPP) gene of the mouse was disrupted by homologous recombination; however, contrary to expectation, brain and other tissues still contained βAPP-specific RNA, albeit at a level 5-10 fold lower than wild-type and lacking the disrupted exon, which had been spliced out. The brain contained shortened βAPP-specific protein at a low level. Mutant mice were severely impaired in spatial learning and exploratory behavior and showed increased incidence of agenesis of the corpus callosum.

Electronic Resource

1420-9071

Key words. Prion diseases; neurografts; BSE; scrapie; transgenic mice; knockout mice; spongiform encephalopathies.

Springer Online Journal Archives 1860-2000

Biology

Medicine

Abstract. The physical nature of the agent that causes transmissible spongiform encephalopathies (the 'prion'), is the subject of passionate controversy. Investigation of it has benefited tremendously from the use of transgenic and knockout technologies. However, prion diseases present several other enigmas, including the mechanism of brain damage and how the affinity of the agent for the central nervous system is controlled. Here we show that such questions can be effectively addressed in transgenic and knockout systems, and that pathogenesis may be clarified even before we can be certain about the nature of the infectious agent. Availability of mice overexpressing the Prnp gene (which encodes the normal prion protein) and Prnp knockout mice allows for selective reconstitution experiments aimed at expressing PrP in specific portions of the brain or in selected populations of hemato- and lymphopoietic origin. We summarize how such studies can offer insights into how prions administered to peripheral sites can gain access to central nervous tissue, and into the molecular requirements for spongiform brain damage.

Electronic Resource

1432-1440

Neurodegeneration ; Transgenic mouse ; Prions ; Spumavirinae ; Alzheimer's disease ; Anryotrophic lateral sclerosis

Springer Online Journal Archives 1860-2000

Medicine

Abstract Accurate animal models are essential for detailed analysis of the mechanisms underlying human neurodegenerative diseases. In addition, they can offer useful paradigms for the development and evaluation of new therapeutic strategies. We review the most popular techniques for modification of the mammalian genome in vivo, and provide a critical evaluation of the available transgenic mouse models for several neurological conditions of humans, including prion diseases, human retroviral diseases, Alzheimer's disease, and motor neuron diseases.

Electronic Resource

1432-1440

Key words Neurodegeneration ; Transgenic mouse ; Prions ; Spumavirinae ; Alzheimer’s disease ; Amyotrophic lateral sclerosis

Springer Online Journal Archives 1860-2000

Medicine

Abstract  Accurate animal models are essential for detailed analysis of the mechanisms underlying human neurodegenerative diseases. In addition, they can offer useful paradigms for the development and evaluation of new therapeutic strategies. We review the most popular techniques for modification of the mammalian genome in vivo, and provide a critical evaluation of the available transgenic mouse models for several neurological conditions of humans, including prion diseases, human retroviral diseases, Alzheimer’s disease, and motor neuron diseases.

Electronic Resource

1432-0533

Key words Progressive multifocal leukoencephalopathy ; JC virus immunohistochemistry ; Recombinant protein ; In situ hybridization

Springer Online Journal Archives 1860-2000

Medicine

Abstract We have assessed the diagnostic efficacy of a novel polyclonal rabbit antiserum directed to the recombinant major capsid protein VP1 of JC virus (JCV). Immunohistochemistry for VP1 was compared to non-radioactive JCV DNA in situ hybridization (ISH) in ten cases of progressive multifocal leukoencephalopathy (PML). Tissue sections from postmortem brains were studied from PML patients suffering from immunodeficient conditions of various causes: immunodeficiency syndrome (AIDS, n = 7), severe combined immune deficiency due to adenosine deaminase deficiency (n = 1), sarcoidosis (n = 1) and leukemia (n = 1). VP1 immunohistochemistry demonstrated the presence of JCV in lesional oligodendrocytes of all PML patients, whereas ISH was able to detect JCV in nine out of ten cases. We conclude that VP1 immunohistochemistry is a specific, sensitive and rapid method for confirming the diagnosis of PML.

Electronic Resource

1432-0533

Gliosarcoma ; Immunohistochemistry ; Histiocyte

Springer Online Journal Archives 1860-2000

Medicine

Summary Gliosarcomas contain both neuro-ectodermal and mesenchymal elements. Its histogenesis has been much debated and endothelial and adventitial fibroblast origins have been suggested, as has a “histiocytic” origin following the demonstration of antiprotease activity. Eight gliosarcomas have been examined with a panel of ten monoclonal and polyclonal antibodies to investigate the origin of the sarcomatous element. Glial fibrillary acid protein expression showed a sharp distinction between gliomatous and sarcomatous tumour components. Contrary to some previous reports factor 8-related antigen and Ulex europeus agglutinin stained vascular luminal endothelium but no tumour cells. Vimentin and fibronectin expression was extensive and confined largely to sarcomatous areas. Desmin and neurofilament protein could not be demonstrated in any of the cases. Numerous cells, particularly in the sarcoma areas, expressed alpha-1-antitrypsin and-chymotrypsin. A proportion of these stained for the monocyte/macrophage marker MAC 387. Four cases focally exhibited a true stori-form pattern and this and the immunohistochemical results suggest analogies with the fibrous histiocytomas. These tumours contain reactive histiocytes but are now thought to be derived from fibroblasts or from pluripotent mesenchymal cells in perivascular adventitia. This resembles the pattern exhibited in the sarcomatous component of gliosarcomas.

Electronic Resource

1432-0533

Germ cell tumor ; Syncytiotrophoblastic giant cells ; Germinoma ; Embryonal carcinoma ; Precocious puberty ; Ki-67

Springer Online Journal Archives 1860-2000

Medicine

Summary A 9-year-old male patient developed a germ cell tumor in the right basal ganglia which secretedβ-human chorionic gonadotropin (β-HCG) and caused precocious puberty. Histology and immunohistochemical staining for placental alkaline phosphatase (PLAP), α-fetoprotein (α-FP), andβ-HCG showed a mixed population of neoplastic germinocytes, embryonal carcinoma, and syncytiotrophoblastic giant cells (STGC). Immunohistochemical double-staining for α-FP andβ-HCG revealed that these two markers were produced by different subsets of cells. Expression of the proliferation marker Ki-67 showed a growth fraction of 53% for the neoplastic germinocytes and embryonal carcinoma cells, but only 21% for the STGC.

Electronic Resource

0942-0940

Medulloblastoma ; recurrence ; metastases

Springer Online Journal Archives 1860-2000

Medicine

Summary Although primary treatment of medulloblastoma is now successful in a high percentage of patients, its secondary manifestations still bear a poor prognosis. Thorough studies of secondary manifestations are therefore pivotal to plan therapeutic approaches for the long-term management of medulloblastoma. Here we describe the incidence of secondary tumour manifestations in 66 patients of a single centre who underwent surgery for medulloblastoma between 1975 and 1990. No patient was excluded due to a poor postoperative course. Thirty-five patients showed evidence of secondary tumour growth. Of these, 17 suffered from local recurrence, and 27 developed metastastatic disease. The median latencies for secondary manifestations were 25 months for local recurrence (n = 17), 11 months for spinal metastases (n = 10), 15 months for supratentorial metastases (n = 8), 8 months for subleptomeningeal dissemination (n = 6), and 23 months for systemic metastases (n = 8). Two patients developed primary metastatic spread to the posterior fossa. Of 8 patients with supratentorial metastases, 6 developed fronto-basal lesions. In our patients, 89% of secondary lesions occurred within less than 3 years after primary diagnosis. 85% of patients with extra-axial tumour spread had been treated with a permanent shunt. Radical tumour resection and radiotherapy with 30 Gy to the neuraxis and 20 Gy boost to the posterior fossa was an important prognostic factor in this series. Patients with additional chemotherapy did not benefit significantly from this treatment. We conclude that optimal management of the primary lesions should aim at (i) total resection, (ii) avoid permanent shunting, and (iii) completion of the radiotherapy with inclusion of the medial frontobasal cisterns in the radiotherapeutic regimen. Our analysis suggests that adequate postoperative screening programmes should consist of 3-monthly scans of the neuraxis in the first three postoperative years and 6-monthly scans thereafter.

Electronic Resource