نتایج جستجو برای: fanconi anemia

تعداد نتایج: 58591  

1997

GC, Grompe M: Germ cell defects and hematopoietic sensitivity to 11. Poot M, Gross O, Epe B, Pflaum M, Hoehn H: Cell cycle g-interferon in mice with a targeted disruption of the Fanconi anemia defect in connection with oxygen and iron sensitivity in Fanconi C gene. Blood 88:49, 1996 anemia lymphoblastoid cells. Exp Cell Res 222:262, 1996 2. Saito H, Hammond AT, Moses RE: Hypersensitivity to oxy...

Journal: :Clinical cancer research : an official journal of the American Association for Cancer Research 2005
Michiel S van der Heijden Jonathan R Brody David A Dezentje Eike Gallmeier Steven C Cunningham Michael J Swartz Angelo M DeMarzo G Johan A Offerhaus William H Isacoff Ralph H Hruban Scott E Kern

PURPOSE BRCA2, FANCC, and FANCG gene mutations are present in a subset of pancreatic cancer. Defects in these genes could lead to hypersensitivity to interstrand cross-linkers in vivo and a more optimal treatment of pancreatic cancer patients based on the genetic profile of the tumor. EXPERIMENTAL DESIGN Two retrovirally complemented pancreatic cancer cell lines having defects in the Fanconi ...

Journal: :Journal of the National Cancer Institute 2008
Hester J T van Zeeburg Peter J F Snijders Thijs Wu Eliane Gluckman Jean Soulier Jordi Surralles Maria Castella Jacqueline E van der Wal Johan Wennerberg Joseph Califano Eunike Velleuer Ralf Dietrich Wolfram Ebell Elisabeth Bloemena Hans Joenje C René Leemans Ruud H Brakenhoff

Fanconi anemia is a recessively inherited disease that is characterized by congenital abnormalities, bone marrow failure, and a predisposition to develop cancer, particularly squamous cell carcinomas (SCCs) in the head and neck and anogenital regions. Previous studies of Fanconi anemia SCCs, mainly from US patients, revealed the presence of high-risk human papillomavirus (HPV) DNA in 21 (84%) o...

Journal: :Haematologica 2016
Antonio M Risitano Serena Marotta Rita Calzone Francesco Grimaldi Adriana Zatterale

The natural history of Fanconi anemia remains hard to establish because of its rarity and its heterogeneous clinical presentation; since 1994, the Italian Fanconi Anemia Registry has collected clinical, epidemiological and genetic data of Italian Fanconi Anemia patients. This registry includes 180 patients with a confirmed diagnosis of Fanconi anemia who have either been enrolled prospectively,...

Journal: :Cancer discovery 2015
Sarah L Sawyer Lei Tian Marketta Kähkönen Jeremy Schwartzentruber Martin Kircher Jacek Majewski David A Dyment A Micheil Innes Kym M Boycott Lisa A Moreau Jukka S Moilanen Roger A Greenberg

UNLABELLED Deficiency in BRCA-dependent DNA interstrand crosslink (ICL) repair is intimately connected to breast cancer susceptibility and to the rare developmental syndrome Fanconi anemia. Bona fide Fanconi anemia proteins, BRCA2 (FANCD1), PALB2 (FANCN), and BRIP1 (FANCJ), interact with BRCA1 during ICL repair. However, the lack of detailed phenotypic and cellular characterization of a patient...

2012
Younghoon Kee Min Huang Sophia Chang Lisa A. Moreau Eunmi Park Peter G. Smith Alan D. D'Andrea

The Fanconi anemia pathway is required for repair of DNA interstrand cross-links (ICL). Fanconi anemia pathway–deficient cells are hypersensitive to DNA ICL–inducing drugs such as cisplatin. Conversely, hyperactivation of the Fanconi anemia pathway is a mechanism that may underlie cellular resistance to DNA ICL agents. Modulating FANCD2 monoubiquitination, a key step in the Fanconi anemia pathw...

Journal: :Clinical genetics 2011
M H Kang

SLX4, a coordinator of structure-specific endo-nucleases, is mutated in a new Fanconi anemia subtype Stoepker et al. (2011) Nature Genetics 43:138-141. Mutations of the SLX4 gene in Fanconi anemia Kim et al. (2011) Nature Genetics 43:142-146.

Journal: :Cell 2015
Anderson T. Wang Agata Smogorzewska

Fanconi anemia is a genetic disorder resulting from biallelic mutations in one of the 17 FANC genes. It is characterized by congenital abnormalities, bone marrow failure, and cancer predisposition. The underlying cause is genomic instability resulting from the deficiency in replication-dependent DNA interstrand crosslink repair pathway commonly referred to as the Fanconi anemia-BRCA pathway. Th...

Journal: :Current Biology 2017

Journal: :the archives of bone and joint surgery 0
ahmadreza afshar department of orthopedics, imam khomeini hospital, urmia university of medical sciences, urmia, iran

this case report presents a case of fanconi’s anemia with an unusual thumb polydactyly in a 2-year old boy. the extra thumb had no nail, nail bed and distal phalanx. the extra thumb had no active motion.the duplication of the thumb occurred at the carpometacarpal joint but its morphology did not match with any classification described for thumb polydactyly. although his thumb polydactyly was ap...

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