Sebastian Brandner

University College London, Centre for Stem Cells and Regenerative Medicine, Faculty Member

Followers

Following

Co-author

Public Views

see websites

http://www.ion.ucl.ac.uk/divisions/neuropathology.htm
UCL Neurosciences
http://www.ucl.ac.uk/neuroscience/Page.php?ID=12&ResearcherID=189
MRC Neuromuscular Centre (UCL)
http://www.cnmd.ac.uk/researchgroups/sbrandner
University College London Hospitals
http://www.uclh.nhs.uk/GPs+healthcare+professionals/Consultants/Prof+Sebastian+Brandner.htm
Address: Division of Neuropathology and
Dept. of Neurodegenerative Disease
UCL Institute of Neurology
Queen Square
London WC1N 3BG
United Kingdom

less

Ghent University

Ghent University

UCLouvain (University of Louvain)

Interests

Uploads

Papers by Sebastian Brandner

Germline SDHD mutation in paraganglioma of the spinal cord

by Dov Soffer and Sebastian Brandner

Oncogene, 2001

Download

Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.

The EMBO …, Jan 1, 1996

Download

Normal host prion protein necessary for scrapie-induced neurotoxicity

Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions

Cell, Jan 1, 1998

Download

Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis

Science, Jan 1, 2003

Mutations In the Endosomal ESCRTIII-Complex Subunit CHMP2B In Frontotemporal Dementia

Nature genetics, Jan 1, 2005

Monoclonal antibodies inhibit prion replication and delay the development of prion disease

Nature, Jan 1, 2003

Shared allelic losses on chromosomes 1p and 19q suggest a common origin of oligodendroglioma and oligoastrocytoma

… of Neuropathology & …, Jan 1, 1995

Behavioral and Anatomical Deficits In Mice Homozygous for a Modified [Beta]-Amyloid Precursor Protein Gene

Cell, Jan 1, 1994

PrP-Expressing Tissue Required for Transfer of Scrapie Infectivity From Spleen to Brain

Nature, Jan 1, 1997

Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report

The Lancet, Jan 1, 2006

Neuroprotective Role of the Reaper-Related Serine Protease HtrA2/Omi Revealed by Targeted Deletion In Mice

… and cellular biology, Jan 1, 2004

Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system

Proceedings of the …, Jan 1, 1996

An aneuploid mouse strain carrying human chromosome 21 with Down syndrome phenotypes

Science, Jan 1, 2005

Astrocyte-specific expression of hamster prion protein (PrP) renders PrP knockout mice susceptible to hamster scrapie

The EMBO …, Jan 1, 1997

Transmissible spongiform encephalopathies are characterized by spongiosis, astrocytosis and accum... more Transmissible spongiform encephalopathies are characterized by spongiosis, astrocytosis and accumulation of PrP Sc , an isoform of the normal host protein PrP C . The exact cell types responsible for agent propagation and pathogenesis are still uncertain. To determine the possible role of astrocytes, we generated mice devoid of murine PrP but expressing hamster PrP transgenes driven by the astrocyte-specific GFAP promoter. After inoculation with hamster scrapie, these mice accumulated infectivity and PrP Sc to high levels, developed severe disease after 227 Ϯ 5 days and died 7 Ϯ 4 days later. Therefore, astrocytes could play an important role in scrapie pathogenesis, possibly by an indirect toxic effect on neurons. Interestingly, mice expressing the same transgenes but also endogenous murine PrP genes propagated infectivity without developing disease. Keywords: astrocytes/hamster scrapie prions/PrP/ transgenic mice

Download

Human prion protein with valine 129 prevents expression of variant CJD phenotype

Science, Jan 1, 2004

Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice

Neuron, Jan 1, 2007

Currently, no treatment can prevent the cognitive and motor decline associated with widespread ne... more Currently, no treatment can prevent the cognitive and motor decline associated with widespread neurodegeneration in prion disease. However, we previously showed that targeting endogenous neuronal prion protein (PrP C ) (the precursor of its disease-associated isoform, PrP Sc ) in mice with early prion infection reversed spongiform change and prevented clinical symptoms and neuronal loss. We now show that cognitive and behavioral deficits and impaired neurophysiological function accompany early hippocampal spongiform pathology. Remarkably, these behavioral and synaptic impairments recover when neuronal PrP C is depleted, in parallel with reversal of spongiosis. Thus, early functional impairments precede neuronal loss in prion disease and can be rescued. Further, they occur before extensive PrP Sc deposits accumulate and recover rapidly after PrP C depletion, supporting the concept that they are caused by a transient neurotoxic species, distinct from aggregated PrP Sc . These data suggest that early intervention in human prion disease may lead to recovery of cognitive and behavioral symptoms.

Download

Ectopic expression of prion protein (PrP) in T lymphocytes or hepatocytes of PrP knockout mice is insufficient to sustain prion replication

Proceedings of the …, Jan 1, 1999

Combined thalidomide and temozolomide treatment in patients with glioblastoma multiforme

Journal of neuro- …, Jan 1, 2004

Porphobilinogen deaminase deficiency in mice causes a neuropathy resembling that of human hepatic porphyria

Nature genetics, Jan 1, 1996

Germline SDHD mutation in paraganglioma of the spinal cord

by Dov Soffer and Sebastian Brandner

Oncogene, 2001

Download

Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.