نتایج جستجو برای: monosomy x

تعداد نتایج: 624158  

2018
Victor B. Pastor Sushree S. Sahoo Jessica Boklan Georg C. Schwabe Ebru Saribeyoglu Brigitte Strahm Dirk Lebrecht Matthias Voss Yenan T. Bryceson Miriam Erlacher Gerhard Ehninger Marena Niewisch Brigitte Schlegelberger Irith Baumann John C. Achermann Akiko Shimamura Jochen Hochrein Ulf Tedgård Lars Nilsson Henrik Hasle Melanie Boerries Hauke Busch Charlotte M. Niemeyer Marcin W. Wlodarski

Familial myelodysplastic syndromes arise from haploinsufficiency of genes involved in hematopoiesis and are primarily associated with early-onset disease. Here we describe a familial syndrome in seven patients from four unrelated pedigrees presenting with myelodysplastic syndrome and loss of chromosome 7/7q. Their median age at diagnosis was 2.1 years (range, 1-42). All patients presented with ...

Journal: :Blood 2016
Marcin W Wlodarski Shinsuke Hirabayashi Victor Pastor Jan Starý Henrik Hasle Riccardo Masetti Michael Dworzak Markus Schmugge Marry van den Heuvel-Eibrink Marek Ussowicz Barbara De Moerloose Albert Catala Owen P Smith Petr Sedlacek Arjan C Lankester Marco Zecca Victoria Bordon Susanne Matthes-Martin Jonas Abrahamsson Jörn Sven Kühl Karl-Walter Sykora Michael H Albert Bartlomiej Przychodzien Jaroslaw P Maciejewski Stephan Schwarz Gudrun Göhring Brigitte Schlegelberger Annámaria Cseh Peter Noellke Ayami Yoshimi Franco Locatelli Irith Baumann Brigitte Strahm Charlotte M Niemeyer

Germline GATA2 mutations cause cellular deficiencies with high propensity for myeloid disease. We investigated 426 children and adolescents with primary myelodysplastic syndrome (MDS) and 82 cases with secondary MDS enrolled in 2 consecutive prospective studies of the European Working Group of MDS in Childhood (EWOG-MDS) conducted in Germany over a period of 15 years. Germline GATA2 mutations a...

2008
TERRY MAGNUSON SANDRA SMITH C. J. EPSTEIN

In general, autosomal monosomy is lethal much earlier in mammalian development than autosomal trisomy. In an attempt to understand why monosomy is so deleterious, we have begun to characterize the development of mouse embryos monosomic for chromosome 19. A dramatic loss of monosomy 19 embryos was found to occur between days 3 and 4 of development. This loss occurred both in vivo and in vitro an...

2014
A Hashemi MH Sheikhha MA Manouchehri SM Kalantar

BACKGROUND Monosomy is defined as the presence of only one chromosome instead of two in humans. Partial monosomy occurs when only a portion of the chromosome is present in a single copy, while the rest has two copies. It can occur in unbalanced translocations or deletions. CASE REPORT In this report, a 6 years old girl was presented who was referred to the Pediatric Dep, Shahid Sadoughi Hospi...

Journal: :Clinical cancer research : an official journal of the American Association for Cancer Research 2007
Lori A Worley Michael D Onken Erica Person Diane Robirds Julie Branson Devron H Char Arie Perry J William Harbour

PURPOSE To compare a gene expression-based classifier versus the standard genetic prognostic marker, monosomy 3, for predicting metastasis in uveal melanoma. EXPERIMENTAL DESIGN Gene expression profiling, fluorescence in situ hybridization (FISH), and array comparative genomic hybridization (aCGH) were done on 67 primary uveal melanomas. Clinical and pathologic prognostic factors were also as...

2010
Nina Larsson Rolf Billström Henrik Lilljebjörn Carin Lassen Johan Richter Marja Ekblom Thoas Fioretos

This is an author produced version of a paper published in Cancer Genetics and Cytogenetics. This paper has been peer-reviewed but does not include the final publisher proof-corrections or journal pagination. "Genetic analysis of dasatinib-treated chronic myeloid leukemia rapidly developing into acute myeloid leukemia with monosomy 7 in Philadelphia-negative cells." Abstract Despite the recent ...

Journal: :Hypertension 2002
Jeffrey P Gardner Xiao-Yan Yang Joan Skurnick Patricia D Wilson Hana Aviv Smita Patel Amy L Davidow Michael Gutkin Abraham Aviv

This work explores the notion that low-frequency, acquired aneuploidy may play a role in complex genetic traits such as essential hypertension. To this end, renal epithelial cells in urinary sediments and in renal cysts were examined by fluorescent in situ hybridization with DNA probes specific for the heterochromatic and centromere regions of chromosomes 16 and 1. Chromosome 16 was probed beca...

2012
Luize Otero Daiane Correa de Souza Rita de Cássia Tavares Bernadete Evangelho Gomes Telma França Padilha Luiz Fernando Bouzas Teresa de Souza Fernandez Eliana Abdelhay

Monosomy 7 arises as a recurrent chromosome aberration in donor cell leukemia after hematopoietic stem cell transplantation. We report a new case of donor cell leukemia with monosomy 7 following HLA-identical allogenic bone marrow transplantation for severe aplastic anemia (SAA). The male patient received a bone marrow graft from his sister, and monosomy 7 was detected only in the XX donor cell...

Journal: :Journal of medical genetics 1992
M M Lai P N Scriven C Ball A C Berry

We report a case of monosomy for the distal region of the short arm of chromosome 10 (p13----ter) associated with trisomy for the terminal region of the long arm of chromosome 5 (q35.2----ter) that had originated from adjacent 1 segregation of a maternal reciprocal balanced translocation (5;10)(q35.2;p13). We review the clinical findings of previously reported cases of both partial monosomy for...

Journal: :Journal of medical genetics 1985
J L Watt I A Olson A W Johnston H S Ross D A Couzin G S Stephen

A family in which a pericentric inversion of chromosome 22, inv(22)(p11q12), is segregating is described. Special reference is made to a unique recombinant subject with a 'pure' partial monosomy 22 syndrome of maternal origin. An attempt has been made to correlate the phenotypic abnormalities with monosomy for the segment 22q12----qter.

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