Molecular genetics of familial tumour syndromes of the central nervous system
Although most of the central nervous system tumours are sporadic, rarely they are associated with familial tumour syndromes. These disorders usually present with an autosomal dominant inheritance and neoplasia develops at younger age than in sporadic cases. Most of these tumours are bilateral, multiplex or multifocal. The causative mutations occur in genes involved in cell cycle regulation, cell growth, differentiation and DNA repair. Studying these hereditary cancer predisposition syndromes associated with nervous system tumours can facilitate the deeper understanding of the molecular background of sporadic tumours and the development of novel therapeutic agents. This review is an update on hereditary tumour syndromes with nervous system involvement with emphasis on molecular genetic characteristics and their clinical implications.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2015 |
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| Erschienen: |
2015 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:156 |
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| Enthalten in: |
Orvosi hetilap - 156(2015), 5 vom: 01. Feb., Seite 171-7 |
| Sprache: |
Ungarisch |
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| Weiterer Titel: |
A központi idegrendszert érintő családi daganatszindrómák molekuláris háttere |
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| Beteiligte Personen: |
Murnyák, Balázs [VerfasserIn] |
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| Links: |
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| Themen: |
Cancer syndrome |
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| Anmerkungen: |
Date Completed 24.04.2015 Date Revised 25.11.2016 published: Print Citation Status MEDLINE |
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| doi: |
10.1556/OH.2015.30092 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
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| 520 | |a Although most of the central nervous system tumours are sporadic, rarely they are associated with familial tumour syndromes. These disorders usually present with an autosomal dominant inheritance and neoplasia develops at younger age than in sporadic cases. Most of these tumours are bilateral, multiplex or multifocal. The causative mutations occur in genes involved in cell cycle regulation, cell growth, differentiation and DNA repair. Studying these hereditary cancer predisposition syndromes associated with nervous system tumours can facilitate the deeper understanding of the molecular background of sporadic tumours and the development of novel therapeutic agents. This review is an update on hereditary tumour syndromes with nervous system involvement with emphasis on molecular genetic characteristics and their clinical implications | ||
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