نتایج جستجو برای: germline mutation

تعداد نتایج: 300136  

Journal: :Gut 2004
N J H Sturt M C Gallagher P Bassett C R Philp K F Neale I P M Tomlinson A R J Silver R K S Phillips

BACKGROUND Many patients with familial adenomatous polyposis (FAP) die from desmoid tumours which can arise spontaneously but often appear to be surgically induced by prophylactic colectomy. FAP results from germline adenomatous polyposis coli (APC) gene mutations and desmoids arise following biallelic APC mutation, with one change usually occurring distal to the second beta-catenin binding/deg...

2004
N J H Sturt P M Tomlinson A R J Silver R K S Phillips

Background: Many patients with familial adenomatous polyposis (FAP) die from desmoid tumours which can arise spontaneously but often appear to be surgically induced by prophylactic colectomy. FAP results from germline adenomatous polyposis coli (APC) gene mutations and desmoids arise following biallelic APC mutation, with one change usually occurring distal to the second b-catenin binding/degra...

Journal: :Journal of medical genetics 2001
R B van der Luijt P H van Zon R P Jansen C J van der Sijs-Bos C C Wárlám-Rodenhuis M G Ausems

Germline mutations in either of the two major breast cancer predisposition genes, BRCA1 and BRCA2, account for a significant proportion of hereditary breast/ovarian cancer. Identification of breast cancer patients carrying mutations of these genes is primarily based on a positive family history of breast/ovarian cancer or early onset of the disease or both. In the course of mutation screening o...

Journal: :Human molecular genetics 2016
Joseph A Toonen Corina Anastasaki Laura J Smithson Scott M Gianino Kairong Li Robert A Kesterson David H Gutmann

Neurofibromatosis type 1 (NF1) is a common neurogenetic condition characterized by significant clinical heterogeneity. A major barrier to developing precision medicine approaches for NF1 is an incomplete understanding of the factors that underlie its inherent variability. To determine the impact of the germline NF1 gene mutation on the optic gliomas frequently encountered in children with NF1, ...

Journal: :Clinical genetics 2012
M A Shah E Salo-Mullen Z Stadler J M Ruggeri M Mirander Y Pristyazhnyuk L Zhang

In this report, we describe the first concluded case of a de novo germline mutation in CDH1 in a hereditary diffuse gastric cancer (HDGC) kindred. The incident case was a woman with a personal history of Hodgkin's lymphoma and diffuse gastric cancer, who was then confirmed to have a CDH1 mutation (c.1792 C>T (R598X)). The patient's mother was found to have the same CDH1 germline mutation; howev...

2015
Hongan Long David J. Winter Allan Y-C. Chang Way Sung Steven H. Wu Mariel Balboa Ricardo B. R. Azevedo Reed A. Cartwright Michael Lynch Rebecca A. Zufall

Mutation is the ultimate source of all genetic variation and is, therefore, central to evolutionary change. Previous work on the ciliate Paramecium tetraurelia concluded that the presence of a transcriptionally silent germline genome has caused the evolution of a low base-substitution mutation rate in the germline genome of that ciliate. Here, we use mutation accumulation (MA) lines of the cili...

2010
Zehra Aycan Sebahat Yılmaz Ağladıoğlu Serdar Ceylaner Semra Çetinkaya Veysel Nijat Baş Havva Nur Peltek Kendirici

Neonatal hyperthyroidism is a rare disorder and occurs in two forms. An autoimmune form is associated with maternal Graves' disease, resulting from transplacental passage of maternal thyroid-stimulating antibodies and a nonautoimmune form is caused by gain of function mutations in the thyrotropin receptor (TSHR) gene. Thyrotoxicosis caused by germline mutations in the TSHR gene may lead to a va...

2015
Marianne S. Elston Goswin Y. Meyer-Rochow Michael Dray Michael Swarbrick John V. Conaglen

Individuals presenting with primary hyperparathyroidism (PHPT) at a young age commonly have an underlying germline gene mutation in one of the following genes: MEN1, CASR, or CDC73. A small number of families with primary hyperparathyroidism have been identified with germline mutations in CDKN1B and those patients with primary hyperparathyroidism have almost exclusively been women who present i...

Journal: :American journal of human genetics 2004
Sakari Vanharanta Mary Buchta Sarah R McWhinney Sanna K Virta Mariola Peçzkowska Carl D Morrison Rainer Lehtonen Andrzej Januszewicz Heikki Järvinen Matti Juhola Jukka-Pekka Mecklin Eero Pukkala Riitta Herva Maija Kiuru Nina N Nupponen Lauri A Aaltonen Hartmut P H Neumann Charis Eng

Hereditary paraganglioma syndrome has recently been shown to be caused by germline heterozygous mutations in three (SDHB, SDHC, and SDHD) of the four genes that encode mitochondrial succinate dehydrogenase. Extraparaganglial component neoplasias have never been previously documented. In a population-based registry of symptomatic presentations of phaeochromocytoma/paraganglioma comprising 352 re...

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