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PRODID:talks.ox.ac.uk
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SUMMARY:Nuclear mechanical collapse of Purkinje neurons in Ataxia-telangie
 ctasia - Dr Conor Lowndes (FIRC Institute of Molecular Oncology)
DTSTART;VALUE=DATE-TIME:20250723T120000
DTEND;VALUE=DATE-TIME:20250723T130000
UID:https://new.talks.ox.ac.uk/talks/id/39b6f301-ac61-4d84-b216-da751a60ef
 54/
DESCRIPTION:Ataxia-telangiectasia (A-T) is a neurodegenerative disorder ca
 used by ATM mutations\, leading to progressive Purkinje neuron loss. While
  ATM regulates the DNA damage response\, mechanisms of cell degeneration r
 emain unclear. Using cerebella\, hiPS cells\, and cerebellar organoids fro
 m A-T patients\, we show that Purkinje cells shift from a soft\, low-visco
 sity state to hyperstiffness with nuclear deformation and chromatin hyperm
 ethylation. Proteomics reveal dysregulated mechanotransduction\, chromatin
  remodelling\, and loss of calcium channels. Epigenetic inhibition restore
 s nuclear structure and ITPR1 levels. These findings identify A-T as a “
 mechano-disease” and suggest that targeting nuclear mechanics could offe
 r new therapeutic avenues\nSpeakers:\nDr Conor Lowndes (FIRC Institute of 
 Molecular Oncology)
LOCATION:Kennedy Institute of Rheumatology (Kennedy Lecture Theatre)\, Hea
 dington OX3 7FY
TZID:Europe/London
URL:https://new.talks.ox.ac.uk/talks/id/39b6f301-ac61-4d84-b216-da751a60ef
 54/
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DESCRIPTION:Talk:Nuclear mechanical collapse of Purkinje neurons in Ataxia
 -telangiectasia - Dr Conor Lowndes (FIRC Institute of Molecular Oncology)
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