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111.
Amy L. Schneider Candace T. Myers Alison M. Muir Sophie Calvert Alice Basinger M. Scott Perry Lance Rodan Katherine L. Helbig Chelsea Chambers Kathleen M. Gorman Mary D. King Sandra Donkervoort Ariane Soldatos Carsten G. Bnnemann Nino Spataro Elisabeth Gabau Montserrat Arellano Gerarda Cappuccio Nicola Brunetti‐Pierri Elsa Rossignol Fadi F. Hamdan Jacques L. Michaud Christopher Balak Heather C. Mefford Ingrid E. Scheffer 《Epilepsia》2021,62(1):e13-e21
Chromosome 1q41‐q42 deletion syndrome is a rare cause of intellectual disability, seizures, dysmorphology, and multiple anomalies. Two genes in the 1q41‐q42 microdeletion, WDR26 and FBXO28, have been implicated in monogenic disease. Patients with WDR26 encephalopathy overlap clinically with those with 1q41‐q42 deletion syndrome, whereas only one patient with FBXO28 encephalopathy has been described. Seizures are a prominent feature of 1q41‐q42 deletion syndrome; therefore, we hypothesized that pathogenic FBXO28 variants cause developmental and epileptic encephalopathies (DEEs). We describe nine new patients with FBXO28 pathogenic variants (four missense, including one recurrent, three nonsense, and one frameshift) and analyze all 10 known cases to delineate the phenotypic spectrum. All patients had epilepsy and 9 of 10 had DEE, including infantile spasms (3) and a progressive myoclonic epilepsy (1). Median age at seizure onset was 22.5 months (range 8 months to 5 years). Nine of 10 patients had intellectual disability, which was profound in six of nine and severe in three of nine. Movement disorders occurred in eight of 10 patients, six of 10 had hypotonia, four of 10 had acquired microcephaly, and five of 10 had dysmorphic features, albeit different to those typically seen in 1q41‐q42 deletion syndrome and WDR26 encephalopathy. We distinguish FBXO28 encephalopathy from both of these disorders with more severe intellectual impairment, drug‐resistant epilepsy, and hyperkinetic movement disorders. 相似文献
112.
David G. Pearson Catherine Deeprose Sophie M.A. Wallace-Hadrill Stephanie Burnett Heyes Emily A. Holmes 《Clinical psychology review》2013
Mental imagery is an under-explored field in clinical psychology research but presents a topic of potential interest and relevance across many clinical disorders, including social phobia, schizophrenia, depression, and post-traumatic stress disorder. There is currently a lack of a guiding framework from which clinicians may select the domains or associated measures most likely to be of appropriate use in mental imagery research. We adopt an interdisciplinary approach and present a review of studies across experimental psychology and clinical psychology in order to highlight the key domains and measures most likely to be of relevance. This includes a consideration of methods for experimentally assessing the generation, maintenance, inspection and transformation of mental images; as well as subjective measures of characteristics such as image vividness and clarity. We present a guiding framework in which we propose that cognitive, subjective and clinical aspects of imagery should be explored in future research. The guiding framework aims to assist researchers in the selection of measures for assessing those aspects of mental imagery that are of most relevance to clinical psychology. We propose that a greater understanding of the role of mental imagery in clinical disorders will help drive forward advances in both theory and treatment. 相似文献
113.
Emilie Montellier Fay?al Boussouar Sophie Rousseaux Kai Zhang Thierry Buchou Fran?ois Fenaille Hitoshi Shiota Alexandra Debernardi Patrick Héry Sandrine Curtet Mahya Jamshidikia Sophie Barral Hélène Holota Aurélie Bergon Fabrice Lopez Philippe Guardiola Karin Pernet Jean Imbert Carlo Petosa Minjia Tan Yingming Zhao Matthieu Gérard Saadi Khochbin 《Genes & development》2013,27(15):1680-1692
The conversion of male germ cell chromatin to a nucleoprotamine structure is fundamental to the life cycle, yet the underlying molecular details remain obscure. Here we show that an essential step is the genome-wide incorporation of TH2B, a histone H2B variant of hitherto unknown function. Using mouse models in which TH2B is depleted or C-terminally modified, we show that TH2B directs the final transformation of dissociating nucleosomes into protamine-packed structures. Depletion of TH2B induces compensatory mechanisms that permit histone removal by up-regulating H2B and programming nucleosome instability through targeted histone modifications, including lysine crotonylation and arginine methylation. Furthermore, after fertilization, TH2B reassembles onto the male genome during protamine-to-histone exchange. Thus, TH2B is a unique histone variant that plays a key role in the histone-to-protamine packing of the male genome and guides genome-wide chromatin transitions that both precede and follow transmission of the male genome to the egg. 相似文献
114.
115.
Audrey Delas Dobbelstein Sophie Pierre Brousset Camille Laurent 《Pathology, research and practice》2013
We report herein a case of blastoid variant mantle cell lymphoma (MCL) with both aberrant phenotype and unusual genetics. Unexpectedly, lymphoma cells were CD5− and CD10+. Standard karyotype and FISH techniques showed that tumor cells carried two distinct translocations which had not been reported together in a same tumor. The first translocation juxtaposed the immunoglobulin lambda light chain locus with CCND1 locus, leading to Cyclin D1 overexpression. The second translocation revealed MYC rearrangement with a non-immunoglobulin gene partner located on the short arm of chromosome 4. The interpretation of the case on tissue sections alone could have been challenging. Indeed, the lack of CD5 and expression of CD10 associated with MYC rearrangement detected on interphasic nuclei could support the diagnosis of diffuse large B-cell lymphoma or Burkitt lymphoma. This distinction is also especially important as these lymphoma subtypes require specific treatment. 相似文献
116.
M. Laura Feltri Michael R. Weaver Sophie Belin Yannick Poitelon 《Journal of the peripheral nervous system : JPNS》2021,26(1):4-16
Initially identified in Drosophila, the Hippo signaling pathway regulates how cells respond to their environment by controlling proliferation, migration and differentiation. Many recent studies have focused on characterizing Hippo pathway function and regulation in mammalian cells. Here, we present a brief overview of the major components of the Hippo pathway, as well as their regulation and function. We comprehensively review the studies that have contributed to our understanding of the Hippo pathway in the function of the peripheral nervous system and in peripheral nerve diseases. Finally, we discuss innovative approaches that aim to modulate Hippo pathway components in diseases of the peripheral nervous system. 相似文献
117.
Rogier Thomas Eberl Isabelle Moretto Florian Sixt Thibault Catherine François-Xavier Estève Clémentine Abdallahoui Maroua Behague Lucile Coussement Antoine Mathey Lucas Mahy Sophie Buisson Marielle Salmon-Rousseau Arnaud Duong Michel Chavanet Pascal Bernard Quentin Nicolas Barbara Benguella Leila Bonnotte Bernard Blot Mathieu Piroth Lionel 《European journal of clinical microbiology & infectious diseases》2021,40(9):2023-2028
European Journal of Clinical Microbiology & Infectious Diseases - During an epidemic period, we compared patients hospitalized for initial suspicion of COVID-19 but for whom an alternative... 相似文献
118.
119.
Baron Sophie Alexandra Pascale Léa-Marie Million Matthieu Briantais Antoine Durand Jean-Marc Hadjadj Linda Rolain Jean-Marc 《European journal of clinical microbiology & infectious diseases》2021,40(5):1073-1077
European Journal of Clinical Microbiology & Infectious Diseases - We described three clinical cases of pyogenic liver abscess caused by hypervirulent Klebsiella pneumoniae (hvKp) successfully... 相似文献
120.
Klug Tejs Ehlers Greve Thomas Andersen Camilla Hahn Pernille Danstrup Christian Petersen Niels Krintel Ninn-Pedersen Mirjana Mikkelsen Sophie Pauli Søren Fuglsang Simon Døssing Helle Christensen Anne-Louise Rusan Maria Kjeldsen Anette 《European journal of clinical microbiology & infectious diseases》2021,40(7):1461-1470
European Journal of Clinical Microbiology & Infectious Diseases - We aimed to describe the microbiology of parapharyngeal abscess (PPA) and point out the likely pathogens using the following... 相似文献