Nguyen Hiep Tai, Tang Weiliang, Webster Andrew L H, Whiteaker Jeffrey R, Chandler Christopher M, Errazquin Ricardo, Roohollahi Khashayar, Fritzke Madeline, Hoskins Elizabeth E, Jonlin Erica, Wakefield Leslie, Sullivan Lucas B, Chen Eleanor Y, Dorsman Josephine, Brakenhoff Ruud, Paulovich Amanda G, Grompe Markus, Garcia-Escudero Ramon, Wells Susanne I, Smogorzewska Agata, Monnat Raymond J
Department of Laboratory Medicine and Pathology, University of Washington, Seattle, Washington, USA.
Laboratory of Genome Maintenance, Rockefeller University, New York, New York, USA.
Int J Cancer. 2023 Jul 1;153(1):183-196. doi: 10.1002/ijc.34506. Epub 2023 Mar 30.
Fanconi anemia (FA) is a heritable malformation, bone marrow failure and cancer predisposition syndrome that confers an exceptionally high risk of squamous carcinomas. These carcinomas originate in epithelia lining the mouth, proximal esophagus, vulva and anus: their origins are not understood, and no effective ways have been identified to prevent or delay their appearance. Many FA-associated carcinomas are also therapeutically challenging: they may be multi-focal and stage-advanced at diagnosis, and most individuals with FA cannot tolerate standard-of-care systemic therapies such as DNA cross-linking drugs or ionizing radiation due to constitutional DNA damage hypersensitivity. We developed the Fanconi Anemia Cancer Cell Line Resource (FA-CCLR) to foster new work on the origins, treatment and prevention of FA-associated carcinomas. The FA-CCLR consists of Fanconi-isogenic head and neck squamous cell carcinoma (HNSCC) cell line pairs generated from five individuals with FA-associated HNSCC, and five individuals with sporadic HNSCC. Sporadic, isogenic HNSCC cell line pairs were generated in parallel with FA patient-derived isogenic cell line pairs to provide comparable experimental material to use to identify cell and molecular phenotypes driven by germline or somatic loss of Fanconi pathway function, and the subset of these FA-dependent phenotypes that can be modified, complemented or suppressed. All 10 FANC-isogenic cell line pairs are available to academic, non-profit and industry investigators via the "Fanconi Anemia Research Materials" Resource and Repository at Oregon Health & Sciences University, Portland OR.
范可尼贫血(FA)是一种遗传性畸形、骨髓衰竭和癌症易感性综合征,具有极高的鳞状细胞癌风险。这些癌症起源于口腔、近端食管、外阴和肛门的上皮组织:其起源尚不清楚,且尚未找到有效的预防或延缓其出现的方法。许多与FA相关的癌症在治疗上也具有挑战性:它们在诊断时可能是多灶性且处于晚期,并且由于先天性DNA损伤超敏反应,大多数FA患者无法耐受标准的全身治疗,如DNA交联药物或电离辐射。我们开发了范可尼贫血癌细胞系资源(FA - CCLR),以促进对与FA相关癌症的起源、治疗和预防的新研究。FA - CCLR由来自5名患有FA相关头颈部鳞状细胞癌(HNSCC)的个体以及5名患有散发性HNSCC的个体所产生的范可尼同基因HNSCC细胞系对组成。与FA患者来源的同基因细胞系对并行产生散发性、同基因HNSCC细胞系对,以提供可比的实验材料,用于识别由范可尼途径功能的种系或体细胞缺失驱动的细胞和分子表型,以及这些FA依赖性表型中可被修饰、补充或抑制的子集。所有10个FANC同基因细胞系对可通过俄勒冈健康与科学大学(位于俄勒冈州波特兰)的“范可尼贫血研究材料”资源库提供给学术、非营利和行业研究人员。