نتایج جستجو برای: microdeletion

تعداد نتایج: 1516  

Journal: :Molecular syndromology 2010
M van Kogelenberg S Ghedia G McGillivray D Bruno R Leventer K Macdermot J Nelson L Nagarajan J A Veltman A P de Brouwer R J McKinlay Gardner H van Bokhoven E P Kirk S P Robertson

Periventricular heterotopia (PH) is a brain malformation characterised by heterotopic nodules of neurons lining the walls of the cerebral ventricles. Mutations in FLNA account for 20-24% of instances but a majority have no identifiable genetic aetiology. Often the co-occurrence of PH with a chromosomal anomaly is used to infer a new locus for a Mendelian form of PH. This study reports four PH p...

2015
Lukrecija Brečević Martina Rinčić Željka Krsnik Goran Sedmak Ahmed B. Hamid Nadezda Kosyakova Ivan Galić Thomas Liehr Fran Borovečki

We describe an as yet unreported neocentric small supernumerary marker chromosome (sSMC) derived from chromosome 1p21.3p21.2. It was present in 80% of the lymphocytes in a male patient with intellectual disability, severe speech deficit, mild dysmorphic features, and hyperactivity with elements of autism spectrum disorder (ASD). Several important neurodevelopmental genes are affected by the 3.5...

2015
Aram Yang Yeon Hee Lee Soon Young Nam Yu Ju Jeong Yechan Kyung Rimm Huh Jieun Lee Younghee Kwun Sung Yoon Cho Dong-Kyu Jin

PURPOSE Prader-Willi syndrome (PWS) is a well-known genetic disorder, and microdeletion on chromosome 15 is the most common causal mechanism. Several previous studies have suggested that various environmental factors might be related to the pathogenesis of microdeletion in PWS. In this study, we investigated birth seasonality in Korean PWS. METHODS A total of 211 PWS patients born from 1980 t...

Journal: :Molecular syndromology 2012
M Zweier A Rauch

Disorders related to the autosomal transcription factor MEF2C located in 5q14.3 were first described in 2009 and have since evolved to one of the more common microdeletion syndromes. Mutational screening in a larger cohort revealed heterozygous de novo mutations of MEF2C in about 1% of patients with moderate to severe intellectual disability, and the phenotype is similar in patients with intrag...

Journal: :The American Journal of Human Genetics 2003

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