نتایج جستجو برای: megacystis

تعداد نتایج: 120  

Journal: :Human molecular genetics 2016
Danny Halim Robert M W Hofstra Luca Signorile Rob M Verdijk Christine S van der Werf Yunia Sribudiani Rutger W W Brouwer Wilfred F J van IJcken Niklas Dahl Joke B G M Verheij Clarisse Baumann John Kerner Yolande van Bever Niels Galjart Rene M H Wijnen Dick Tibboel Alan J Burns Françoise Muller Alice S Brooks Maria M Alves

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare congenital disorder, in which heterozygous missense variants in the Enteric Smooth Muscle actin γ-2 (ACTG2) gene have been recently identified. To investigate the mechanism by which ACTG2 variants lead to MMIHS, we screened a cohort of eleven MMIHS patients, eight sporadic and three familial cases, and performed immunoh...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1999
W Xu S Gelber A Orr-Urtreger D Armstrong R A Lewis C N Ou J Patrick L Role M De Biasi A L Beaudet

The alpha3 subunit of the neuronal nicotinic acetylcholine receptor is widely expressed in autonomic ganglia and in some parts of the brain. The alpha3 subunit can form heteromultimeric ion channels with other alpha subunits and with beta2 and beta4 subunits, but its function in vivo is poorly understood. We prepared a null mutation for the alpha3 gene by deletion of exon 5 and found that homoz...

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