RRC ID 31344
Author Nakada Y, Taniura H, Uetsuki T, Inazawa J, Yoshikawa K.
Title The human chromosomal gene for necdin, a neuronal growth suppressor, in the Prader-Willi syndrome deletion region.
Journal Gene
Abstract Necdin is a growth suppressor expressed in virtually all postmitotic neurons in the brain. The human necdin gene, NDN, is maternally imprinted and deleted in the Prader-Willi syndrome, a neurobehavioral contiguous gene disorder. Here, we isolated and characterized the human chromosomal necdin gene and its promoter region. The necdin gene is intronless, and it encodes a protein of 321 amino acid residues, four residues shorter than mouse Necdin. By fluorescence in-situ hybridization analysis, the necdin gene was localized to chromosome 15q11.2-q12 within the Prader-Willi syndrome deletion region. CpG islands were found in a region extending from the proximal 5'-flanking sequence to the protein coding region. The 5'-flanking sequence, which lacks canonical TATA and CAAT boxes, possessed a promoter activity in postmitotic neurons derived from murine embryonal carcinoma P19 cells. Methylation in vitro of HhaI CpG sites in the promoter region reduced the transcriptional activity. These results suggest that the necdin gene is silenced through methylation of the CpG island encompassing its promoter region.
Volume 213(1-2)
Pages 65-72
Published 1998-6-15
DOI 10.1016/s0378-1119(98)00206-6
PII S0378-1119(98)00206-6
PMID 9630521
MeSH Amino Acid Sequence Animals Base Sequence Carcinoma, Embryonal / pathology CpG Islands DNA Methylation Female Genes* Genomic Imprinting Humans Hypothalamus / metabolism In Situ Hybridization Mice Molecular Sequence Data Nerve Tissue Proteins / genetics* Neurons / metabolism Nuclear Proteins / genetics* Prader-Willi Syndrome / genetics* Promoter Regions, Genetic Sequence Alignment Sequence Deletion* Sequence Homology Species Specificity Transcription, Genetic Tumor Cells, Cultured
IF 2.984
Times Cited 50
DNA material pcHNDN (RDB02986).