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SUMMARY:TLR3-IFN deficiencies underlying Herpes simplex encephalitis - Dr 
 Vanessa Sancho-Simizu\, Imperial College London
DTSTART:20121010T113000Z
DTEND:20121010T123000Z
UID:TALK39762@talks.cam.ac.uk
CONTACT:Sue Griffin
DESCRIPTION:Herpes simplex encephalitis (HSE) is a rare complication of he
 rpes simplex virus-1 (HSV-1) infection affecting young children as well as
  adults. Although rare in occurrence\, HSE remains the most common cause o
 f acute\, sporadic viral encephalitis in the western world.   HSE patients
  are otherwise healthy with no apparent immunodeficiency or increased susc
 eptibly to other viral infections or other forms of HSV-1 infections.  \n\
 nWe have hypothesized that at least a subset of patients with HSE\, in par
 ticular children\, are genetically predisposed to HSE due to a specific im
 munodeficiency in their response to HSV-1 infection.  We have now identifi
 ed mutations in five genes of the TLR3-IFN pathway leading to impaired TLR
 3-dependent induction of interferons in patients with HSE. These patients 
 are unable to control HSV1 replication in fibroblasts however their antivi
 ral response in PBMCs are normal\, consistent with the CNS-restricted and 
 HSV-1-restricted infectious phenotype in these children\, who are otherwis
 e normally resistant to other infections.   \n\nHSE provides proof-of-prin
 ciple that a collection of single-gene variations displaying incomplete pe
 netrance at the clinical level and affecting different\, but immunological
 ly related genes\, may account for severe\, sporadic and common infectious
  diseases of childhood. 
LOCATION:Lecture Theatre\, Department of Pathology\, Tennis Court Road
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