نتایج جستجو برای: myh9 gene

تعداد نتایج: 1141526  

Journal: :American journal of nephrology 2010
Barry I Freedman Jeffrey C Edberg Mary E Comeau Mariana Murea Donald W Bowden Jasmin Divers Graciela S Alarcón Elizabeth E Brown Gerald McGwin Jeffrey B Kopp Cheryl A Winkler George W Nelson Gabor Illei Michelle Petri Rosalind Ramsey-Goldman John D Reveille Luis M Vilá Carl D Langefeld Robert P Kimberly

BACKGROUND African Americans (AA) disproportionately develop lupus nephritis (LN) relative to European Americans and familial clustering supports causative genes. Since MYH9 underlies approximately 40% of end-stage renal disease (ESRD) in AA, we tested for genetic association with LN. METHODS Seven MYH9 single nucleotide polymorphisms (SNPs) and the E1 risk haplotype were tested for associati...

2016
P. Canzi A. Pecci M. Manfrin E. Rebecchi C. Zaninetti V. Bozzi M. Benazzo

MYH9-related disease (MYH9-RD) is a rare genetic syndromic disorder characterised by congenital thrombocytopenia and is associated with the risk of developing progressive sensorineural hearing loss, nephropathy and presenile cataracts during childhood or adult life. All consecutive patients enrolled in the Italian Registry for MYH9-RD with severe to profound deafness were included in a retrospe...

Journal: :Vojnosanitetski pregled 2014
Milos Kuzmanović Shinji Kunishima Jovana Putnik Natasa Stajić Aleksandra Paripović Radovan Bogdanović

INTRODUCTION The group of autosomal dominant disorders - Epstein syndrome, Sebastian syndrome, Fechthner syndrome and May-Hegglin anomaly - are characterised by thrombocytopenia with giant platelets, inclusion bodies in granulocytes and variable levels of deafness, disturbances of vision and renal function impairment. A common genetic background of these disorders are mutations in MYH9 gene, co...

Journal: :Blood 2009
Anita Eckly Catherine Strassel Monique Freund Jean-Pierre Cazenave François Lanza Christian Gachet Catherine Léon

Mutations in the MYH9 gene encoding nonmuscle myosin IIA lead to macrothrombocytopenia as observed in MYH9-related disorders. We used mice with megakaryocyte-restricted MYH9 inactivation to explore the role of myosin in thrombopoiesis. In situ, bone marrow MYH9Delta megakaryocytes were irregularly shaped, appearing leaky with poorly defined limits. The demarcation membranes were abnormally orga...

2010
Fabio Fabbian Franco Ricci Alfredo De Giorgi Silvia Forcellini Giovanni Scanelli

We report a 37-year-old renal transplanted patient with thrombocytopaenia. He had May–Hegglin anomaly (MHA) inherited from his father, who died due to myocardial infarction subsequent to two renal transplantations. At the age of 15 years, proteinuria was found, but he was not biopsied. In 1982, he underwent splenectomy because of trauma. In 1989, when he was 17, renal function was found to be d...

Journal: :Blood 2015
Kyle R Spinler Jae-Won Shin Michele P Lambert Dennis E Discher

Megakaryocyte ploidy and the generation of pre/proplatelets are both increased in culture by pharmacologic inhibition of myosin-II, but nonmuscle myosin-IIA (MIIA) mutations paradoxically cause MYH9-related diseases (MYH9-RD) that adversely affect platelets. In marrow, megakaryocytes extend projections into the microcirculation, where shear facilitates fragmentation to large pre/proplatelets, s...

2011
Barry I. Freedman Carl D. Langefeld Lingyi Lu Jasmin Divers Mary E. Comeau Jeffrey B. Kopp Cheryl A. Winkler George W. Nelson Randall C. Johnson Nicholette D. Palmer Pamela J. Hicks Meredith A. Bostrom Jessica N. Cooke Caitrin W. McDonough Donald W. Bowden

Single nucleotide polymorphisms (SNPs) in MYH9 and APOL1 on chromosome 22 (c22) are powerfully associated with non-diabetic end-stage renal disease (ESRD) in African Americans (AAs). Many AAs diagnosed with type 2 diabetic nephropathy (T2DN) have non-diabetic kidney disease, potentially masking detection of DN genes. Therefore, genome-wide association analyses were performed using the Affymetri...

Journal: :Kidney Research and Clinical Practice 2015

Journal: :Science 2014
Daniel Schramek Ataman Sendoel Jeremy P Segal Slobodan Beronja Evan Heller Daniel Oristian Boris Reva Elaine Fuchs

Mining modern genomics for cancer therapies is predicated on weeding out "bystander" alterations (nonconsequential mutations) and identifying "driver" mutations responsible for tumorigenesis and/or metastasis. We used a direct in vivo RNA interference (RNAi) strategy to screen for genes that upon repression predispose mice to squamous cell carcinomas (SCCs). Seven of our top hits-including Myh9...

2014
Yanhua Rao Rui Hao Bin Wang Tso-Pang Yao

Primary cilia are specialized, acetylated microtubule-based signaling processes. Cilium assembly is activated by cellular quiescence and requires reconfiguration of microtubules, the actin cytoskeleton, and vesicular trafficking machinery. How these components are coordinated to activate ciliogenesis remains unknown. Here we identify the microtubule acetyltransferase Mec-17 and myosin II motors...

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