نتایج جستجو برای: hereditary sensory and autonomic neuropathies

تعداد نتایج: 16848591  

2013
Aniket Ghosh Tina Kling Nicolas Snaidero Julio L. Sampaio Andrej Shevchenko Heribert Gras Bart Geurten Martin C. Göpfert Jörg B. Schulz Aaron Voigt Mikael Simons

Glia are of vital importance for all complex nervous system. One of the many functions of glia is to insulate and provide trophic and metabolic support to axons. Here, using glial-specific RNAi knockdown in Drosophila, we silenced 6930 conserved genes in adult flies to identify essential genes and pathways. Among our screening hits, metabolic processes were highly represented, and genes involve...

2017
Martha Smets Stephanie Link Patricia Wolf Katrin Schneider Veronica Solis Joel Ryan Daniela Meilinger Weihua Qin Heinrich Leonhardt

DNMT1 is recruited to substrate sites by PCNA and UHRF1 to maintain DNA methylation after replication. The cell cycle dependent recruitment of DNMT1 is mediated by the PCNA-binding domain (PBD) and the targeting sequence (TS) within the N-terminal regulatory domain. The TS domain was found to be mutated in patients suffering from hereditary sensory and autonomic neuropathies with dementia and h...

Journal: :Brain : a journal of neurology 2002
Jean-Philippe Camdessanché Jean-Christophe Antoine Jérôme Honnorat Christophe Vial Philippe Petiot Philippe Convers Daniel Michel

Although paraneoplastic subacute sensory neuronopathy is the most frequent presentation of peripheral neuropathy in patients with anti-Hu antibodies, other neuropathies have been reported. In order to investigate the clinical and electrophysiological manifestations of neuropathies associated with anti-Hu antibodies, we conducted a retrospective study of 20 patients. For the electrophysiological...

Journal: :Medicina oral, patologia oral y cirugia bucal 2008
Martin Romero Rogelio Simón Jose Ignacio García-Recuero Ana Romance

Hereditary sensory and autonomic neuropathy type IV is a rare disease characterized by fever episodes, mental retardation of different intensity, recurrent episodes of fever secondary to anhidrosis, little or no perspiration and congenital insensitivity to pain. Oral self-mutilation is also a characteristic sign. In this article, we present the case of an infant, aged 22 months, who showed thes...

2012
Juan D. Ramirez Phillip R.J. Barnes Kerry R. Mills David L.H. Bennett

We report an English kindred affected across 4 generations with a hereditary neuropathy associated with debilitating neuropathic pain as the main clinical feature. The principal finding on clinical examination was sensory loss, and there was variable motor dysfunction. Electrophysiological studies revealed mild features of demyelination with median conduction velocity in the intermediate range....

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