Novel Human Stem Cell-based Model of Amyotrophic Lateral Sclerosis Opens Doors for Rapid Drug Screening

piggy submitted, created time 1 month 1 day (www.sciencedaily.com)

Long thought of as mere bystanders, astrocytes are crucial for the survival and well-being of motor neurons, which control voluntary muscle movements. In fact, defective astrocytes can lay waste to motor neurons and are the main suspects in the muscle-wasting disease amyotrophic lateral sclerosis (ALS), commonly known as Lou Gherig's disease.

To get to the root of this complicated relationship, researchers from the Salk Institute for Biological Studies for the very first time established a human embryonic stem cell (hESC)-based system for modeling ALS

Submit comment | Blog It | Email It | Keywords: stem cells stem cell research Lou Gherig's disease ALS drug screening SOD1 motor neurons neurons ALS amyotrophic lateral sclerosis astrocytes

SMN Deficiency Causes Tissue-Specific Perturbations in the Repertoire of snRNAs and Widespread Defects in Splicing

sea-maid submitted, created time 7 months 3 weeks (www.cell.com)

With regards to the survival of motor neurons (SMN), certain proteins are essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery.

This article suggests that SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing.

Submit comment | Blog It | Email It | Keywords: motor neurons neurons splicing RNA SMN snRNA small nuclear RNA pre-mRNA splicing