Article
Hsp27 decreases inclusion body formation from mutated GTP-cyclohydrolase I protein.
Biochimica et biophysica acta - 1 Mar 2008
Chiou Yu-Wei, Hwu Wuh-Liang, Lee Yu-May
Abstract excerpt
GTP cyclohydrolase I (GCH), an oligomeric protein composed of 10 identical subunits, is required for the synthesis of neurotransmitters; mutations in GCH are associated with dopa-responsive dystonia (DRD) and hyperphenylalaninemia. Mutated GCH proteins are unstable and prone to dominant-negative effect. We show herein that expression of the GCH mutant GCH-201E or the splicing variant GCH-II caused intracellular...
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