نتایج جستجو برای: uniparental disomy

تعداد نتایج: 1450  

Journal: :Journal of medical genetics 2002
M Rio F Molinari S Heuertz C Ozilou P Gosset O Raoul V Cormier-Daire J Amiel S Lyonnet M Le Merrer C Turleau M-C de Blois M Prieur S Romana M Vekemans A Munnich L Colleaux

Recent studies have shown that cryptic unbalanced subtelomeric rearrangements contribute to a significant proportion of idiopathic syndromic mental retardation cases. Using a fluorescent genotyping based strategy, we found a 10% rate of cryptic subtelomeric rearrangements in a large series of 150 probands with severe idiopathic syndromic mental retardation and normal RHG-GTG banded karyotype. F...

2017
Nicole G. Griffin Kenneth D. Cronin Nicole M. Walley Christine M. Hulette Gerald A. Grant Mohamad A. Mikati Heather G. LaBreche Catherine W. Rehder Andrew S. Allen Peter B. Crino Erin L. Heinzen

Hemimegalencephaly (HME) is a heterogeneous cortical malformation characterized by enlargement of one cerebral hemisphere. Somatic variants in mammalian target of rapamycin (mTOR) regulatory genes have been implicated in some HME cases; however, ∼70% have no identified genetic etiology. Here, we screened two HME patients to identify disease-causing somatic variants. DNA from leukocytes, buccal ...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2012
Joseph L Roberts Rebecca H Buckley Biao Luo Jianming Pei Alla Lapidus Suraj Peri Qiong Wei Jinwook Shin Roberta E Parrott Roland L Dunbrack Joseph R Testa Xiao-Ping Zhong David L Wiest

Analysis of the molecular etiologies of SCID has led to important insights into the control of immune cell development. Most cases of SCID result from either X-linked or autosomal recessive inheritance of mutations in a known causative gene. However, in some cases, the molecular etiology remains unclear. To identify the cause of SCID in a patient known to lack the protein-tyrosine phosphatase C...

Journal: :Journal of medical genetics 2003
W M A Verhoeven S Tuinier L M G Curfs

P rader-Willi syndrome (PWS) is a neurogenetic disorder resulting from the absence of a normal paternal contribution to the chromosome 15q11-13 region. The clinical manifestations of PWS are: reduced fetal activity during pregnancy, a transient severe hypotonia and feeding problems in the neonatal period, a variable degree of mental retardation, hyperphagia, obsessive compulsive features such a...

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