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A series of relatively short (GCC)ntriplet repeats (n¼3–30) located within regulatory regions of many mammalian genes may be considered as puta-tive cis-acting transcriptional elements (GCC-elements). Fragile X-mental retardation syndrome is caused by an expansion of (GCC)ntriplet repeats within the 5¢-untranslated region of the human fragile X-mental retarda-tion 1 (FMR1) gene. | ễFEBS Journal Novel repressor of the human FMR1 gene - identification of p56 human GCC n-binding protein as a Kruppel-like transcription factor ZF5 Sergey V. Orlov1 2 Konstantin B. Kuteykin-Teplyakov1 Irina A. Ignatovich3 Ella B. Dizhe1 Olga A. Mirgorodskaya3 Alexander V. Grishin2 Olga B. Guzhova1 Egor B. Prokhortchouk4 Pavel V. Guliy1 2 and Andrej P. Perevozchikov1 2 1 Department of Biochemistry Institute of ExperimentalMedicine Russian Academy of MedicalSciences St Petersburg Russia 2 Department of Embryology St Petersburg State University Russia 3 Institute of Cytology Russian Academy of Sciences St Petersburg Russia 4 Department of Molecular Basis of Medicine Institute of Gene Biology Russian Academy of Sciences Moscow Russia Keywords FMR1 fragile X syndrome GCC n triplet repeats ZF5 zinc finger transcription factors Correspondence S. V. Orlov Department of Biochemistry Institute of ExperimentalMedicine Russian Academy of MedicalSciences 197376 Acad. Pavlov Street 12 St Petersburg Russia Fax 7 812 234 0310 Tel 7 812 346 0644 E-mail serge@iem.sp.ru Present address Department of Molecular Neurobiochemistry Ruhr-University Bochum 44801 Germany Received 1 May 2007 revised 20 July 2007 accepted 24 July 2007 doi 10.1111 j.1742-4658.2007.06006.x A series of relatively short GCC n triplet repeats n 3-30 located within regulatory regions of many mammalian genes may be considered as putative cis-acting transcriptional elements GCC-elements . Fragile X-mental retardation syndrome is caused by an expansion of GCC n triplet repeats within the 5 -untranslated region of the human fragile X-mental retardation 1 FMR1 gene. The present study aimed to characterize a novel human GCC n-binding protein and investigate its possible role in the regulation of the FMR1 gene. A novel human GCC n-binding protein p56 was isolated and identified as a Knippel-like transcription factor ZF5 by MALDI-TOF analysis. The capacity of ZF5 to specifically interact with GCC n triplet repeats was .
