title: Identification of the full-length huntingtin- interacting protein p231HBP/HYPB as a DNA-binding factor.
abstract: Neurodegeneration in Huntington's disease (HD) is associated with an elongated glutamine tract in the widely expressed huntingtin protein. Although the pathogenic mechanisms are still unknown, the distinct physical properties of mutant huntingtin in the brain suggest that other factors including huntingtin-interacting proteins might play a specific role. We have previously identified a DNA-binding motif in the proximal E1A promoter of adenovirus serotype 12 as responsible for E1A autoregulation. Here, we identified the p231HBP protein as a DNA-binding factor, the C-terminal portion of which has recently been characterized as the huntingtin-interacting protein HYPB of unknown function. We have determined the full-length cDNA sequence, identified several domains supporting its gene regulatory functions, and mapped the HBP231 gene to chromosome 3p21.2-p21.3. Our results provide an interesting molecular link between huntingtin and a DNA-binding factor, implicating that this interaction might result in the alteration of cellular gene expression involved in HD pathogenesis.
authors.affiliation: Center for Cancer Research and Cancer Therapy, Department of Internal Medicine, Institute of Molecular Biology, University of Essen, Hufelandstrasse 55, Essen, D-45122, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Rega
authors.forename: T
authors.initials: T
authors.lastname: Stiewe
authors.forename: D I
authors.initials: DI
authors.lastname: Chang
authors.forename: B
authors.initials: B
authors.lastname: Pollmeier
authors.forename: H
authors.initials: H
authors.lastname: Esche
authors.forename: W
authors.initials: W
authors.lastname: Bardenheuer
authors.forename: G
authors.initials: G
authors.lastname: Marquitan
authors.forename: B M
authors.initials: BM
authors.lastname: Pützer
mesh_terms: D017500:Adenovirus E1A Proteins / Q000235:genetics; D000595:Amino Acid Sequence; D000818:Animals; D002874:Chromosome Mapping; D002893:Chromosomes, Human, Pair 3; D018076:DNA, Complementary; D004268:DNA-Binding Proteins / Q000235:genetics* / Q000378:metabolism*; D015870:Gene Expression / Q000502:physiology; D006367:HeLa Cells; D006801:Humans; D000071058:Huntingtin Protein; D017404:In Situ Hybridization, Fluorescence; D008969:Molecular Sequence Data; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D009687:Nuclear Proteins / Q000378:metabolism*; D011401:Promoter Regions, Genetic / Q000502:physiology; D011817:Rabbits; D020798:Two-Hybrid System Techniques
chemical_list: D017500:Adenovirus E1A Proteins; D018076:DNA, Complementary; D004268:DNA-Binding Proteins; C086055:HTT protein, human; D000071058:Huntingtin Protein; C435268:Huntingtin-interacting protein p231HBP; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins