References
De Baere I, Liu L, Moens L, et al. (1992) Polar zipper sequence in the high affinity hemoglobin of Ascaris suum: Amino acid sequence and structural interpretation. Proc. Natl. Acad. Sci. U.S.A. 89: 4638–4642.
Sherman DR, Kloek AP, Krishnan BR, Guinn B, Goldberg DE. (1992) Ascaris hemoglobin gene: Plant-like structure reflects the ancestral globin gene. Proc. Natl. Acad. Sci. U.S.A. 89: 11696–11700.
Perutz MF, Staden R, Moens L, De Baere I. (1993) Polar zippers. Curr. Biol. 3: 249–253.
The Huntington’s Disease Collaborative Group. (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell 72: 971–983.
Li S-H, Schilling G, Young III WS, et al. (1993) Huntington’s disease gene (IT15) is widely expressed in human and rat tissues. Neuron 11: 985.
Sharp AH, Loev SJ, Schilling G, et al. (1995) Widespread expression of Huntington’s disease gene (IT15) protein product. Neuron 14: 1065–1074.
Trottier Y, Devys D, Imbert G, et al. (1995) Cellular localisation of the Huntington’s disease protein and discrimination of the normal and mutated form. Nature Genet. 10: 104.
Persichetti F, Ambrose CM, Ge P, et al. (1995) Normal and expanded Huntington’s disease alleles produce distinguishable proteins due to translation across the CAG repeat. Mol. Med. 1: 374–383.
Ali N, Bienz M. (1991) Functional dissection of abdominal-β protein. Mech. Dev. 35: 55–64.
Courey AJ, Tjian R. (1988) Analysis of SP1 in vivo reveals multiple transcriptional domains, including a novel glutamine-rich motif. Cell 55: 887–898.
Courey AJ, Holtzman DA, Jackson SP, Tjian R. (1989) Synergistic activation by the glutamine-rich 721 domains of human transcription factor SP1. Cell 59: 827–836.
Hoey TH, Weinziere ROJ, Gill G, Chen J-L, Dynlacht BD, Tjian R. (1993) Molecular cloning and functional analysis of Drosophila TAF110 reveal properties expected of coactivators. Cell 72: 247–260.
Gerber HP, Seipel K, Georgiev O, et al. (1994) Transcriptional activation modulated by homopolymeric glutamine and proline stretches. Science 263: 808–811.
Baxendale S, Abdulla S, Elgar G, et al. (1995) Comparative sequence analysis of the human and pufferfish Huntington’s disease genes. Nature Genet. 10: 67–76.
Perutz MF, Johnson T, Suzuki M, Finch JT. (1994) Glutamine repeats as polar zippers: Their possible role in inherited neurodegeneative diseases. Proc. Natl. Acad. Sci. U.S.A. 91: 5355–5358.
Stott K, Blackburn JM, Butler PJG, Perutz M. (1995) Incorporation of glutamine repeats makes protein oligomerize: Implications for neurodegenerative diseases. Proc. Natl. Acad. Sci. U.S.A. 92: 6509–6513.
La Spada AR, Paulson HL, Fishbeck KH. (1994) Trinucleotide expansion in neurological disease. Ann. Neurol. 36: 814–822.
Kawaguchi Y, Okamoto T, Taniwaki M, et al. (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32. Nature Genet. 8: 221–227.
Ambrose CM, Duyao MP, Barnes G, et al. (1995) Structure and expression of the Huntington’s disease gene: Evidence against simple inactivation due to an expanded CAG repeat. Somat. Cell Mol. Genet. 20: 27–38.
Li X-J, Li SH, Sharp AH, et al. A Huntington-associated protein enriched in brain with implications for pathology of Huntington’s Disease. Nature (in press).
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Perutz, M.F. Glutamine Repeats as Polar Zippers: Their Role in Inherited Neurodegenerative Disease. Mol Med 1, 718–721 (1995). https://doi.org/10.1007/BF03401886
Published:
Issue Date:
DOI: https://doi.org/10.1007/BF03401886