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State biomarkers for Machado Joseph disease: Validation, feasibility and responsiveness to change Genet. Mol. Biol.
Furtado,Gabriel Vasata; Oliveira,Camila Maria de; Bolzan,Gabriela; Saute,Jonas Alex Morales; Saraiva-Pereira,Maria Luiza; Jardim,Laura Bannach.
Abstract Machado-Joseph disease (SCA3/MJD) is the most common spinocerebellar ataxia worldwide, and particularly so in Southern Brazil. Due to an expanded polyglutamine at ataxin-3, SCA3/MJD presents a relentless course with no current disease modifying treatment. Clinical scales used to measure SCA3/MJD progression present moderate effect sizes, a major drawback for their use as main outcomes in clinical trials, given the rarity and slow progression of the disease. This limitation might be overcome by finding good surrogate markers. We present here a review of studies on peripheral and neurophysiological markers in SCA3/MJD that can be candidates for state biomarkers. Data on markers already studied were summarized, giving emphasis on validation against...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Biomarkers; Neurophysiology; Machado-Joseph disease; Spinocerebellar ataxia type 3.
Ano: 2019 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1415-47572019000200238
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Controle neuromodulateur et flexibilite d'expression d'un reseau neuronal central: Le systeme pylorique des crustaces decapodes ArchiMer
Nagy, F; Cazalets, J; Moulins, M.
In decapod Crustacea, the rhythmic activity of the pyloric filter is programmed within a network of 14 neurons in the stomatogastric nervous system. The pyloric network activity is underlied by both the endogenous regenerative properties of its neurons and the organization of their synaptic connections. Both factors are controlled by central modulatory neurons. Two of these neurons were identified, the APM neuron, which induces, and the PS neuron, which suppresses, the regenerative properties of pyloric neurons. APM can start and PS can stop the rhythmic pyloric activity, but both of them can also change its expression. Because of this control, the pyloric network can produce several types of motor pattern.
Tipo: Text Palavras-chave: Crustacea; Alimentary organs; Neurons; Neurophysiology.
Ano: 1987 URL: http://archimer.ifremer.fr/doc/1987/acte-1386.pdf
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