نتایج جستجو برای: ژن fhl1

تعداد نتایج: 15963  

Journal: :Human molecular genetics 2014
Colleen E D'Arcy Sandra J Feeney Catriona A McLean Stefan M Gehrig Gordon S Lynch Jaclyn E Smith Belinda S Cowling Christina A Mitchell Meagan J McGrath

Utrophin is a potential therapeutic target for the fatal muscle disease, Duchenne muscular dystrophy (DMD). In adult skeletal muscle, utrophin is restricted to the neuromuscular and myotendinous junctions and can compensate for dystrophin loss in mdx mice, a mouse model of DMD, but requires sarcolemmal localization. NFATc1-mediated transcription regulates utrophin expression and the LIM protein...

Journal: :American journal of human genetics 2008
Catarina M Quinzii Tuan H Vu K Christopher Min Kurenai Tanji Sandra Barral Raji P Grewal Andrea Kattah Pilir Camaño David Otaegui Teruhito Kunimatsu David M Blake Kirk C Wilhelmsen Lewis P Rowland Arthur P Hays Eduardo Bonilla Michio Hirano

Scapuloperoneal (SP) syndrome encompasses heterogeneous neuromuscular disorders characterized by weakness in the shoulder-girdle and peroneal muscles. In a large Italian-American pedigree with dominant SP myopathy (SPM) previously linked to chromosome 12q, we have mapped the disease to Xq26, and, in all of the affected individuals, we identified a missense change (c.365G-->C) in the FHL1 gene e...

Journal: :Cardiovascular research 2008
Dobromir Dobrev Erich Wettwer

Chronic atrial diseases are associated with electrical, mechanical, and structural changes (remodelling) that result from activation of diverse signal transduction pathways. In atrial fibrillation (AF), the high atrial rate abbreviates the atrial action potential duration primarily by reduced ICa,L and increased inward-rectifier Kþ currents like the background current (IK1) and a constitutively...

Journal: :International journal of molecular medicine 2010
Mitsugi Matsumoto Kazumori Kawakami Hideki Enokida Kazuki Toki Ryoichiro Matsuda Takeshi Chiyomaru Kenryu Nishiyama Kazuya Kawahara Naohiko Seki Masayuki Nakagawa

We previously reported a simple technique that combines microarray data from clinical bladder cancer (BC) specimens with those from a BC cell line (BOY) treated with a pharmacologic demethylating agent (5-aza-dC). We focused on the human four-and-a-half LIM domains 1 (FHL1) gene which was selected on the basis of previous microarray data analysis. Because LIM domains provide protein-protein bin...

Journal: :Cancer research 2006
Yongquan Shen Zhenyu Jia Robert G Nagele Hitoshi Ichikawa Gary S Goldberg

Anchorage independence and motility are hallmarks of tumor cell growth. Tumor cell growth and morphology can be normalized by contact with nontransformed cells. The Src tyrosine kinase phosphorylates specific sites on the focal adhesion adaptor protein Crk-associated substrate (Cas) to promote nonanchored cell growth and migration. We studied the effects of Src and Cas on the expression of >14,...

Journal: :Cardiovascular research 2008
Zhenjiang Yang Carrie F Browning Haifa Hallaq Liudmila Yermalitskaya Jan Esker Matthew R Hall Andrew J Link Amy-Joan L Ham Meagan J McGrath Christina A Mitchell Katherine T Murray

AIMS Protein-protein interactions are critical for the normal membrane trafficking, localization, and function of voltage-gated ion channels. In human heart, the Shaker-related voltage-gated K(+) channel KCNA5 alpha-subunit forms the major basis of an atrial-specific, ultra-rapid delayed rectifier K(+) current, I(Kur). We sought to identify proteins that interact with KCNA5 in human atrium and ...

Journal: :Circulation. Cardiovascular genetics 2013
Hana Hartmannova Milos Kubanek Marek Sramko Lenka Piherova Lenka Noskova Katerina Hodanova Viktor Stranecky Anna Pristoupilova Jana Sovova Tomas Marek Jana Maluskova Petr Ridzon Josef Kautzner Helena Hulkova Stanislav Kmoch

BACKGROUND Hypertrophic cardiomyopathy with severe left ventricular diastolic dysfunction has been associated with marked exercise intolerance and poor prognosis. However, molecular pathogenesis of this phenotype remains unexplained in a large proportion of cases. METHODS AND RESULTS We performed whole exome sequencing as an initial genetic test in a large Czech family with 3 males affected b...

Journal: :Molecular and cellular biology 2006
Yu Zhao Kerri B McIntosh Dipayan Rudra Stephan Schawalder David Shore Jonathan R Warner

The ribosomal protein genes of Saccharomyces cerevisiae, responsible for nearly 40% of the polymerase II transcription initiation events, are characterized by the constitutive tight binding of the transcription factor Rap1. Rap1 binds at many places in the yeast genome, including glycolytic enzyme genes, the silent MAT loci, and telomeres, its specificity arising from specific cofactors recruit...

Journal: :The Journal of clinical investigation 2008
Joachim Schessl Yaqun Zou Meagan J McGrath Belinda S Cowling Baijayanta Maiti Steven S Chin Caroline Sewry Roberta Battini Ying Hu Denny L Cottle Michael Rosenblatt Lynn Spruce Arupa Ganguly Janbernd Kirschner Alexander R Judkins Jeffrey A Golden Hans-Hilmar Goebel Francesco Muntoni Kevin M Flanigan Christina A Mitchell Carsten G Bönnemann

Reducing body myopathy (RBM) is a rare disorder causing progressive muscular weakness characterized by aggresome-like inclusions in the myofibrils. Identification of genes responsible for RBM by traditional genetic approaches has been impossible due to the frequently sporadic occurrence in affected patients and small family sizes. As an alternative approach to gene identification, we used laser...

Journal: :Genes & development 2015
Rohit Reja Vinesh Vinayachandran Sujana Ghosh B Franklin Pugh

The 137 ribosomal protein genes (RPGs) of Saccharomyces provide a model for gene coregulation. We examined the positional and functional organization of their regulators (Rap1 [repressor activator protein 1], Fhl1, Ifh1, Sfp1, and Hmo1), the transcription machinery (TFIIB, TFIID, and RNA polymerase II), and chromatin at near-base-pair resolution using ChIP-exo, as RPGs are coordinately reprogra...

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