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Novel BRCA2 pathogenic variant c.5219 T > G; p.(Leu1740Ter) in a consanguineous Senegalese family with hereditary breast cancer.在一个患有遗传性乳腺癌的塞内加尔近亲家庭中发现新型BRCA2致病变体c.5219 T > G;p.(Leu1740Ter)
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本文引用的文献

1
The clinical phenotype of children with Fanconi anemia caused by biallelic FANCD1/BRCA2 mutations.双等位基因 FANCD1/BRCA2 突变导致的范可尼贫血患儿的临床表型。
Pediatr Blood Cancer. 2012 Mar;58(3):462-5. doi: 10.1002/pbc.23168. Epub 2011 May 5.
2
Fanconi anemia: a disorder defective in the DNA damage response.范可尼贫血症:一种 DNA 损伤反应缺陷的疾病。
Int J Hematol. 2011 Apr;93(4):417-424. doi: 10.1007/s12185-011-0777-z. Epub 2011 Feb 18.
3
Susceptibility pathways in Fanconi's anemia and breast cancer.范可尼贫血症和乳腺癌的易感性途径。
N Engl J Med. 2010 May 20;362(20):1909-19. doi: 10.1056/NEJMra0809889.
4
Genetic subtyping of Fanconi anemia by comprehensive mutation screening.通过全面的突变筛查对范可尼贫血进行基因分型。
Hum Mutat. 2008 Jan;29(1):159-66. doi: 10.1002/humu.20625.
5
Emergence of a DNA-damage response network consisting of Fanconi anaemia and BRCA proteins.由范可尼贫血蛋白和乳腺癌易感基因(BRCA)蛋白组成的DNA损伤反应网络的出现。
Nat Rev Genet. 2007 Oct;8(10):735-48. doi: 10.1038/nrg2159. Epub 2007 Sep 4.
6
The BRCA1/2 pathway prevents hematologic cancers in addition to breast and ovarian cancers.除了乳腺癌和卵巢癌,BRCA1/2 通路还能预防血液系统癌症。
BMC Cancer. 2007 Aug 6;7:152. doi: 10.1186/1471-2407-7-152.
7
BRCA2 is required for neurogenesis and suppression of medulloblastoma.BRCA2是神经发生和髓母细胞瘤抑制所必需的。
EMBO J. 2007 Jun 6;26(11):2732-42. doi: 10.1038/sj.emboj.7601703. Epub 2007 May 3.
8
The Fanconi anemia/BRCA pathway: a coordinator of cross-link repair.范可尼贫血/乳腺癌易感基因通路:交联修复的协调者
Exp Cell Res. 2006 Aug 15;312(14):2647-53. doi: 10.1016/j.yexcr.2006.06.014. Epub 2006 Jun 21.
9
Clinical and molecular features associated with biallelic mutations in FANCD1/BRCA2.与FANCD1/BRCA2双等位基因突变相关的临床和分子特征。
J Med Genet. 2007 Jan;44(1):1-9. doi: 10.1136/jmg.2006.043257. Epub 2006 Jul 6.
10
Evidence for an age cutoff greater than 365 days for neuroblastoma risk group stratification in the Children's Oncology Group.儿童肿瘤学组中神经母细胞瘤风险组分层年龄界限大于365天的证据。
J Clin Oncol. 2005 Sep 20;23(27):6459-65. doi: 10.1200/JCO.2005.05.571. Epub 2005 Aug 22.

由于BRCA2基因塞浦路斯奠基者突变纯合性导致的范可尼贫血-D1:一例报告。

Fanconi anemia-D1 due to homozygosity for the BRCA2 gene Cypriot founder mutation: A case report.

作者信息

Loizidou Maria A, Hadjisavvas Andreas, Tanteles George A, Spanou-Aristidou Elena, Kyriacou Kyriacos, Christophidou-Anastasiadou Violetta

机构信息

Department of Electron Microscopy/Molecular Pathology, Cyprus Institute of Neurology and Genetics, Nicosia 1683, Cyprus.

Department of Electron Microscopy/Molecular Pathology, Cyprus Institute of Neurology and Genetics, Nicosia 1683, Cyprus; Cyprus School of Molecular Medicine, Cyprus Institute of Neurology and Genetics, Nicosia 1683, Cyprus.

出版信息

Oncol Lett. 2016 Jan;11(1):471-473. doi: 10.3892/ol.2015.3852. Epub 2015 Nov 2.

DOI:10.3892/ol.2015.3852
PMID:26834852
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4727152/
Abstract

Fanconi anemia (FA) is a rare disorder characterized by multiple congenital malformations, progressive bone marrow failure and susceptibility to malignancies. Biallelic mutations in the breast cancer 2, early onset (BRCA2) gene are responsible for the FA-D1 subgroup, which accounts for ~3% of all the FA cases. Patients with biallelic BRCA2 mutations generally display a more severe phenotype, with earlier onset and increased incidence of leukaemia and other solid tumors, than other patients with FA. In the present report, the first Cypriot patient with FA-D1 is described, which is the fifth case of a homozygote for the same null allele reported thus far, and the third known case of neuroblastoma in association with FA-D1.

摘要

范可尼贫血(FA)是一种罕见的疾病,其特征为多种先天性畸形、进行性骨髓衰竭以及易患恶性肿瘤。乳腺癌2号基因(BRCA2)双等位基因突变导致了FA-D1亚组,该亚组约占所有FA病例的3%。与其他FA患者相比,携带BRCA2双等位基因突变的患者通常表现出更严重的表型,发病更早,白血病和其他实体瘤的发病率更高。在本报告中,描述了首例塞浦路斯FA-D1患者,这是迄今为止报道的同一无效等位基因纯合子的第五例病例,也是已知的第三例与FA-D1相关的神经母细胞瘤病例。