نتایج جستجو برای: ژن pms2

تعداد نتایج: 16271  

Journal: :The Journal of Experimental Medicine 2008
Sophie Péron Ayse Metin Pauline Gardès Marie-Alexandra Alyanakian Eamonn Sheridan Christian Peter Kratz Alain Fischer Anne Durandy

Immunoglobulin (Ig) class switch recombination (CSR) deficiencies are rare primary immunodeficiencies characterized by the lack of switched isotype (IgG/IgA/IgE) production. In some cases, CSR deficiencies can be associated with abnormal somatic hypermutation. Analysis of CSR deficiencies has helped reveal the key functions of CSR-triggering molecules, i.e., CD40L, CD40, and effector molecules ...

Journal: :Voprosy onkologii 2021

Breast cancer (BC) is not a typical manifestation of Lynch syndrome. The existence and extent excessive breast risk in carriers pathogenic mutations the syndrome-associated genes (MLH1, MSH2, MSH6, PMS2) remains an open question. In addition, it known that some neoplasms patients with this syndrome are causally linked to hereditary mutation, arise completely independently defect gene DNA mismat...

Journal: :The Ulster Medical Journal 2005
L. A. Devlin J. H. Price P. J. Morrison

This is an autosomal dominant highly penetrant cancer-susceptibility syndrome causedby germline mutations inone oftheDNAmismatch repair genes, MLH1, MSH2, MSH6, PMS2 and PMS 1. Affected individuals have apredispositionto developing early onset colorectal cancer and endometrial cancer, and less commonly ovarian, small intestine, stomach, biliary tract, pancreatic, brain and uroepithelial tract c...

Journal: :Proceedings of the National Academy of Sciences 1999

Journal: :Oncology reports 2016
Kristina Lagerstedt-Robinson Anna Rohlin Christos Aravidis Beatrice Melin Margareta Nordling Marie Stenmark-Askmalm Annika Lindblom Mef Nilbert

Lynch syndrome caused by constitutional mismatch‑repair defects is one of the most common hereditary cancer syndromes with a high risk for colorectal, endometrial, ovarian and urothelial cancer. Lynch syndrome is caused by mutations in the mismatch repair (MMR) genes i.e., MLH1, MSH2, MSH6 and PMS2. After 20 years of genetic counseling and genetic testing for Lynch syndrome, we have compiled th...

Journal: :The Journal of Experimental Medicine 1999
Nayun Kim Grazyna Bozek James C. Lo Ursula Storb

Somatic hypermutation of Ig genes is probably dependent on transcription of the target gene via a mutator factor associated with the RNA polymerase (Storb, U., E.L. Klotz, J. Hackett, Jr., K. Kage, G. Bozek, and T.E. Martin. 1998. J. Exp. Med. 188:689-698). It is also probable that some form of DNA repair is involved in the mutation process. It was shown that the nucleotide excision repair prot...

2017
Pål Møller Toni Seppälä Inge Bernstein Elke Holinski-Feder Paola Sala D Gareth Evans Annika Lindblom Finlay Macrae Ignacio Blanco Rolf Sijmons Jacqueline Jeffries Hans Vasen John Burn Sigve Nakken Eivind Hovig Einar Andreas Rødland Kukatharmini Tharmaratnam Wouter H de Vos Tot Nederveen Cappel James Hill Juul Wijnen Kate Green Fiona Lalloo Lone Sunde Miriam Mints Lucio Bertario Marta Pineda Matilde Navarro Monika Morak Laura Renkonen-Sinisalo Ian M Frayling John-Paul Plazzer Kirsi Pylvanainen Julian R Sampson Gabriel Capella Jukka-Pekka Mecklin Gabriela Möslein

OBJECTIVE Estimates of cancer risk and the effects of surveillance in Lynch syndrome have been subject to bias, partly through reliance on retrospective studies. We sought to establish more robust estimates in patients undergoing prospective cancer surveillance. DESIGN We undertook a multicentre study of patients carrying Lynch syndrome-associated mutations affecting MLH1, MSH2, MSH6 or PMS2....

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