نتایج جستجو برای: cdls

تعداد نتایج: 139  

2014
Jessica Zuin Vedran Franke Wilfred F. J. van IJcken Antoine van der Sloot Ian D. Krantz Michael I. J. A. van der Reijden Ryuichiro Nakato Boris Lenhard Kerstin S. Wendt

The cohesin complex is crucial for chromosome segregation during mitosis and has recently also been implicated in transcriptional regulation and chromatin architecture. The NIPBL protein is required for the loading of cohesin onto chromatin, but how and where cohesin is loaded in vertebrate cells is unclear. Heterozygous mutations of NIPBL were found in 50% of the cases of Cornelia de Lange Syn...

Journal: :Journal of intellectual disability research : JIDR 2011
K Arron C Oliver J Moss K Berg C Burbidge

BACKGROUND Self-injurious and aggressive behaviours are reported as components of some behavioural phenotypes but there are few studies comparing across syndrome groups. In this study we examined the prevalence of these behaviours and the associated person characteristics in seven genetic syndromes. METHODS Questionnaire data on self-injury and aggression, mood, hyperactivity, autism spectrum...

Journal: :Applied sciences 2022

We consider an optimization problem on the maximal magnitude of angular acceleration output-links a commercially available center-driven linkage system (CDLS) for vehicle wipers windshield. The purpose this is to improve steadiness without weakening its normal function. Thus considered under assumptions that frame fixed links unchanged and input-link rotates at same constant speed with length u...

2011
Robert Szyca Krzysztof Leksowski

Cornelia de Lange syndrome (typus degenerativus amstelodamensis, CdLS, Brachmann syndrome) is a complex, congenital, multi-gene anomaly characterized by mental retardation. Its features include growth inhibition, hirsutism, structural anomalies of the limbs and abnormal development of osseous structures of the face. Independent of the phenotype of the disease, 85% of patients are assumed to hav...

2004
LYNN SAGAN

A theory of the origin of eukaryotic cells ("higher" cells which divide by classical mitosis) is presented. By hypothesis, three fundamental organelles: the mitochondria, the photosynthetic plastids and the (9+2) basal bodies of flagella were themselves once free-living (prokaryotic) cells. The evolution of photosynthesis under the anaerobic conditions of the early atmosphere to form anaerobic ...

2009
Chris Oliver Jenny Sloneem Scott Hall Kate Arron Cornelia de Lange

2009
Shimako Kawauchi Anne L. Calof Rosaysela Santos Martha E. Lopez-Burks Clint M. Young Michelle P. Hoang Abigail Chua Taotao Lao Mark S. Lechner Jeremy A. Daniel Andre Nussenzweig Leonard Kitzes Kyoko Yokomori Benedikt Hallgrimsson Arthur D. Lander

Cornelia de Lange Syndrome (CdLS) is a multi-organ system birth defects disorder linked, in at least half of cases, to heterozygous mutations in the NIPBL gene. In animals and fungi, orthologs of NIPBL regulate cohesin, a complex of proteins that is essential for chromosome cohesion and is also implicated in DNA repair and transcriptional regulation. Mice heterozygous for a gene-trap mutation i...

2017
Beatriz Puisac María-Esperanza Teresa-Rodrigo María Hernández-Marcos Carolina Baquero-Montoya María-Concepción Gil-Rodríguez Torkild Visnes Christopher Bot Paulino Gómez-Puertas Frank J. Kaiser Feliciano J. Ramos Lena Ström Juan Pié

Cornelia de Lange syndrome (CdLS) is a congenital developmental disorder characterized by craniofacial dysmorphia, growth retardation, limb malformations, and intellectual disability. Approximately 60% of patients with CdLS carry a recognizable pathological variant in the NIPBL gene, of which two isoforms, A and B, have been identified, and which only differ in the C-terminal segment. In this w...

2016
Satoshi Hayama Kazuto Ohtaka Yasuhito Shoji Tatsunosuke Ichimura Miri Fujita Naoto Senmaru Satoshi Hirano

BACKGROUND AND OBJECTIVES Factors that contribute to difficult laparoscopic cholecystectomy (LC) in acute cholecystitis (AC) that would affect the performance of early surgery remain unclear. The purpose of this study was to identify such risk factors. METHODS One hundred fifty-four patients who underwent LC for AC were retrospectively analyzed. The patients were categorized into early surger...

2014
María E. Teresa-Rodrigo Juliane Eckhold Beatriz Puisac Andreas Dalski María C. Gil-Rodríguez Diana Braunholz Carolina Baquero María Hernández-Marcos Juan C. de Karam Milagros Ciero Fernando Santos-Simarro Pablo Lapunzina Jolanta Wierzba César H. Casale Feliciano J. Ramos Gabriele Gillessen-Kaesbach Frank J. Kaiser Juan Pié

Cornelia de Lange syndrome (CdLS) is a congenital developmental disorder characterized by distinctive craniofacial features, growth retardation, cognitive impairment, limb defects, hirsutism, and multisystem involvement. Mutations in five genes encoding structural components (SMC1A, SMC3, RAD21) or functionally associated factors (NIPBL, HDAC8) of the cohesin complex have been found in patients...

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