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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2018.1
- 수록면
- 302 - 311 (10page)
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초록· 키워드
오류제보하기
Atypical teratoid rhabdoid tumours (ATRTs) are the most common malignant central nervous system tumours in children ${\leq}1year$ of age and represent approximately 1-2% of all pediatric brain tumours. ATRT is a primarily monogenic disease characterized by the bi-allelic loss of the SMARCB1 gene, which encodes the hSNF5 subunit of the SWI/SNF chromatin remodeling complex. Though conventional dose chemotherapy is not effective in most ATRT patients, high dose chemotherapy with autologous stem cell transplant, radiotherapy and/or intrathecal chemotherapy all show significant potential to improve patient survival. Recent epigenetic and transcriptional studies highlight three subgroups of ATRT, each with distinct clinical and molecular characteristics with corresponding therapeutic sensitivities, including epigenetic targeting, and inhibition of tyrosine kinases or growth/lineage specific pathways.
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등록된 정보가 없습니다.
참고문헌 (69)
참고문헌 신청
Arcaro A / 2007 / Novel role for insulin as an autocrine growth factor for malignant brain tumour cells / Biochem J 406:57~66
Athale UH / 2009 / Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies / J Pediatr Hematol Oncol 31:651~663
Bartelheim K / 2016 / Improved 6-year overall survival in AT/RT-results of the registry study rhabdoid 2007 / Cancer Med 5:1765~1775
Biegel JA / 2006 / Molecular genetics of atypical teratoid/rhabdoid tumors / Neurosurg Focus 20:1~7
Biegel JA / 1999 / Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors / Cancer Res 59:74~79
Athale UH / 2009 / Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies / J Pediatr Hematol Oncol 31:651~663
Bartelheim K / 2016 / Improved 6-year overall survival in AT/RT-results of the registry study rhabdoid 2007 / Cancer Med 5:1765~1775
Biegel JA / 2006 / Molecular genetics of atypical teratoid/rhabdoid tumors / Neurosurg Focus 20:1~7
Biegel JA / 1999 / Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors / Cancer Res 59:74~79
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