Billions of times a day chemical instructions in our genes are translated into proteins – tiny machines that work away in our cells before being broken down and recycled by proteasomes. It’s a beautiful, neat process – but it goes awry in neurodegenerative diseases. Faulty genes can disrupt healthy translation, producing sprawling protein clumps called aggregates, which clog up life inside cells. This computer model is based on 3D cryo-electron tomography of a single aggregate in a neuron. The aggregate’s red ribbon-like curls scoop up and disable the green-coloured proteasomes, leaving neurons unable to break down old or faulty proteins – a hallmark of amyotrophic lateral sclerosis. Different aggregates may create different problems – another type forms hard spindles which leave proteasomes alone, but damage the endoplasmic reticulum, potentially causing the symptoms of Huntington’s disease. Studying these differences helps researchers understand how aggregates work, with a view to blasting them apart with drugs.
BPoD stands for Biomedical Picture of the Day. Managed by the MRC London Institute of Medical Sciences the website aims to engage everyone, young and old, in the wonders of biomedicine. Images are kindly provided for inclusion on this website through the generosity of scientists across the globe.
BPoD is also available in Catalan at www.bpod.cat with translations by the University of Valencia.