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Showing 1 to 3 of 3 for “"Survival Motor Neuron protein (SMN)"”.

  1. Untersuchungen zur Biogenese spleißosomaler UsnRNPs und ihrer Bedeutung für die Pathogenese der SMA

    … (SMA) wird durch den Mangel an funktionellem Survival Motor Neuron Protein (SMN) verursacht. Eine Funktion von SMN liegt in der Biogenese spleißosomaler UsnRNPs (U-rich small nuclear ribonucleoprotein particles). Diese Arbeit zeigt in einem SMA-Modell in Hela-Zellkultur, dass der SMN-Mangel zu …

    wurz-thes Repository record for Untersuchungen zur Biogenese spleißosomaler UsnRNPs und ihrer Bedeutung für die Pathogenese der SMA (opens in a new tab)

  2. Prenatal SMN-dependent defects in translation uncover reversible primary cilia phenotypes in a mouse model of spinal muscular atrophy

    … most patients, SMA is caused by mutations in the survival motor neuron 1 gene (SMN1), resulting in insufficient production of full-length, functional SMN protein. The SMN protein is dynamically regulated during development, where high levels of SMN expression during embryogenesis undergo a …

    edinburgh Repository record for Prenatal SMN-dependent defects in translation uncover reversible primary cilia phenotypes in a mouse model of spinal muscular atrophy (opens in a new tab)

  3. Commonalities between SMA and ALS: investigation of ribosome heterogeneity and translational defects

    … and their association with different proteins, known as ribosome-associated proteins (RAPs). In particular, these proteins can exert a direct role on mRNA selection and translation efficiency. At present, some RNA-binding proteins (RBPs), such as Fragile X Messenger Ribonucleoprotein 1 …

    trento Repository record for Commonalities between SMA and ALS: investigation of ribosome heterogeneity and translational defects (opens in a new tab)