Plenary Session
Chair(s)
  • Amos Etzioni, Israel
  • Andrew R. Gennery, United Kingdom
Room
Bozar
Date
19.09.2019, Thursday
Session Time
16:40 - 18:40
Plenary Session No Topic Needed

MOLECULAR MECHANISMS: ROLE OF ATM IN THE IMMUNE SYSTEM

Lecture Time
16:40 - 17:10
Presenter
  • Malcolm Taylor, United Kingdom
Room
Bozar
Date
19.09.2019, Thursday
Session Time
16:40 - 18:40
Presentation Topic
No Topic Needed

Abstract

Abstract Body

ATM has important roles in regulating DNA DSB repair, cell cycle checkpoint activation and DNA damage-induced apoptotic pathways. Patients with the progressive neurodegenerative disorder ataxia telangiectasia, with biallelic mutation of ATM, show both a cellular and clinical hypersensitivity to ionizing radiation. They also show a variable immunodeficiency; immune abnormalities may include low serum IgA, IgE and selective IgG subtypes, elevated IgM and lymphopenia affecting T cells in particular and decreased immune repertoire diversity, resulting from impaired V(D)J and class switch recombination. There is an increased tendency for sino-pulmonary infections due to the immunodeficiency and pulmonary disease is a major cause of death. The second major cause of death is an increased predisposition to lymphoid tumours. Given the role of physiologically-induced DSBs in promoting somatic recombination in the adaptive immune system, and that a failure to repair DSBs generated during the latter process is known to facilitate lymphoid tumourigenesis, some aspects of the clinical phenotype of A-T correspond with the underlying cellular DNA repair defect. But patients with A-T also show an increased risk of carcinoma including tumours in the gastrointestinal tract, endocrine tumours and female breast cancer in adulthood indicating a wider involvement of ATM in cellular processes.

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Plenary Session No Topic Needed

CANCER IN AT: DATA FROM THE FRENCH NATIONAL REGISTRY

Lecture Time
17:10 - 17:40
Presenter
  • Felipe Suarez, France
Room
Bozar
Date
19.09.2019, Thursday
Session Time
16:40 - 18:40
Presentation Topic
No Topic Needed
Plenary Session No Topic Needed

MULTIDISCIPLINARY TREATMENT IN AT

Lecture Time
17:40 - 18:10
Presenter
  • Jan Loeffen, Netherlands
Room
Bozar
Date
19.09.2019, Thursday
Session Time
16:40 - 18:40
Presentation Topic
No Topic Needed
Plenary Session No Topic Needed

ANGIOGENESIS REVISITED: ROLE AND (THERAPEUTIC) IMPLICATIONS OF ENDOTHELIAL METABOLISM

Lecture Time
18:10 - 18:40
Presenter
  • Peter Carmeliet, Belgium
Room
Bozar
Date
19.09.2019, Thursday
Session Time
16:40 - 18:40
Presentation Topic
No Topic Needed