Concepcion Aguado
Lafora complaint is a rare, fatal form of progressive myoclonus epilepsy characterized by nonstop neurodegeneration with epileptic seizures, characterized by the intracellular accumulation of aberrant polyglucosan grains called Lafora bodies. Several workshop have handed multitudinous substantiation of molecular and cellular differences in neural towel from experimental mouse models deficient in either laforin or malin, two proteins related to the complaint. Oxidative stress, differences in proteostasis, and deregulation of seditious signals are some of the molecular differences underpinning this condition in both KO beast models. Lafora bodies appear beforehand in the beast’s life, but numerous of the forenamed molecular aberrant processes and the consequent neurological symptoms postdate only as creatures age. Then, using small RNAseq and quantitative PCR on brain excerpts from laforin and malin KO manly mice of different periods, we show that two different microRNA species, miR- 155 and miR- 146a, are overexpressed in an age-dependent manner. We also observed altered expression of apparent target genes for each of the microRNAs studied in brain excerpts. These results open the path for a detailed analysis of the molecular consequences of laforin and malin insufficiency in brain towel, as well as the implicit part of miR- 155 and miR- 146a as specific biomarkers of complaint progression in LD.
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