title: Effects of flavonoid quercetin on survival of motor neuron gene expression.
abstract: Spinal muscular atrophy (SMA) is a progressive neurodegenerative disease that results in muscle weakness and atrophy. To attenuate disease severity, drug development studies have been applied mainly to target the Survival of Motor Neuron 2 (SMN2) gene, which is an important modifier of SMA. Although several compounds have been tested, there is still no cure for SMA. In this study, SMN2-inducing effects of quercetin, an abundant flavonoid polyphenol in human diet, was investigated in the fibroblast cell lines of two SMA type I patients. Gene expression studies showed that quercetin upregulates SMN2 mRNA up to fourfold, but not the SMN protein level.
authors.affiliation: Hacettepe University, Faculty of Medicine, Department of Medical Biology, Sıhhiye, Ankara, 06100, Turkey.
authors.forename: Gözde
authors.initials: G
authors.lastname: Uzunallı
authors.forename: Gamze
authors.initials: G
authors.lastname: Bora-Tatar
authors.forename: Didem
authors.initials: D
authors.lastname: Dayangaç-Erden
authors.forename: Hayat
authors.initials: H
authors.lastname: Erdem-Yurter
mesh_terms: D000975:Antioxidants / Q000494:pharmacology; D015153:Blotting, Western; D002460:Cell Line; D006801:Humans; D009046:Motor Neurons / Q000166:cytology / Q000187:drug effects / Q000378:metabolism; D011794:Quercetin / Q000494:pharmacology*; D012333:RNA, Messenger / Q000378:metabolism; D055540:Survival of Motor Neuron 2 Protein / Q000235:genetics / Q000378:metabolism; D015854:Up-Regulation / Q000187:drug effects*
chemical_list: D000975:Antioxidants; D012333:RNA, Messenger; C526477:SMN2 protein, human; D055540:Survival of Motor Neuron 2 Protein; D011794:Quercetin
keywords: quercetin; spinal muscular atrophy; survival of motor neuron gene