Plant Pigment May Help Infants with Rare Muscle Disorder

Plant Pigment May Help Infants with Rare Muscle Disorder

Quercetin, a chemical found in herbs, vegetables, grains and certain fruits, may offer a potential therapy for spinal muscular atrophy (SMA), also known as floppy baby syndrome.

SMA is a genetic neuromuscular disorder that affects an estimated 1 in 6,000 infants, leaving them with little or no control of muscle movements. Sadly, those most severely afflicted usually die by the age of two. There is currently no cure. However, the results of this study, which were published in the Journal of Clinical Investigation, indicates that a plant pigment called quercetin may offer an effective therapy in the early stages of the disease.

SMA is driven by a gene mutation that results in the progressive damage to motor neurons, which prevents nerve signaling from the brain and spinal cord to the muscles. According to scientists at the University of Edinburgh, the gene mutation also interferes with the normal process of purging undesirable molecules from cells. Specifically, the researchers point to a buildup of a molecule called beta-catenin. Quercetin, according to the scientists, appears to target and regulate this molecule and improve nerve and muscle cell function.

Reference

Thomas M. Wishart, Chantal A. Mutsaers, Markus Riessland, Michell M. Reimer, Gillian Hunter, Marie L. Hannam, Samantha L. Eaton, Heidi R. Fuller, Sarah L. Roche, Eilidh Somers, Robert Morse, Philip J. Young, Douglas J. Lamont, Matthias Hammerschmidt, Anagha Joshi, Peter Hohenstein, Glenn E. Morris, Simon H. Parson, Paul A. Skehel, Thomas Becker, Iain M. Robinson, Catherina G. Becker, Brunhilde Wirth, Thomas H. Gillingwater. Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy. Journal of Clinical Investigation, 2014; DOI: 10.1172/JCI71318

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