다음과 같은 기능을 한번의 로그인으로 사용 할 수 있습니다.
NTIS 바로가기
Neuron, v.21 no.6, 1998년, pp.1339 - 1351
Chiesa, R. (Department of Cell Biology and, Physiology, Washington University, School of Medicine, , Missouri 63110,, St. Louis, USA) , Piccardo, P. , Ghetti, B. , Harris, D.A.
AI-Helper
Familial prion diseases are caused by mutations in the gene encoding the prion protein (PrP). We have produced transgenic mice that express the mouse homolog of a mutant human PrP containing a nine octapeptide insertion associated with prion dementia. These mice exhibit a slowly progressive neurolog...
J. Virol Bessen 66 2096 1992 10.1128/JVI.66.4.2096-2101.1992 Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent
J. Virol Bolton 65 3667 1991 10.1128/JVI.65.7.3667-3675.1991 Molecular location of a species-specific epitope on the hamster scrapie agent protein
Genet. Anal. Biomol. Eng Borchelt 13 159 1996 10.1016/S1050-3862(96)00167-2 A vector for expressing foreign genes in the brains and hearts of transgenic mice
Nature Bueler 356 577 1992 10.1038/356577a0 Normal development and behavior of mice lacking the neuronal cell-surface PrP protein
Neurology Capellari 49 133 1997 10.1212/WNL.49.1.133 Familial prion disease with a novel 144-bp insertion in the prion protein gene in a Basque family
J. Biol. Chem Caughey 266 18217 1991 10.1016/S0021-9258(18)55257-1 The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive
J. Biol. Chem Chen 270 19173 1995 10.1074/jbc.270.32.19173 Truncated forms of the human prion protein in normal brain and in prion diseases
Nat. Med Chen 3 1009 1997 10.1038/nm0997-1009 Allelic origin of the abnormal prion protein isoform in familial prion diseases
Neurobiol. Aging Chiesa 19 S300 1998 A transgenic mouse model of a familial prion disease with an insertional mutation
Science Cohen 264 530 1994 10.1126/science.7909169 Structural clues to prion replication
Brain Collinge 115 687 1992 10.1093/brain/115.3.687 Inherited prion disease with 144 base pair gene insertion
Nature Collinge 383 685 1996 10.1038/383685a0 Molecular analysis of prion strain variation and the aetiology of `new variant’ CJD
J. Biol. Chem Daude 272 11604 1997 10.1074/jbc.272.17.11604 Identification of intermediate steps in the conversion of a mutant prion protein to a scrapie-like form in cultured cells
Proc. Natl. Acad. Sci. USA Donne 94 13452 1997 10.1073/pnas.94.25.13452 Structure of the recombinant full-length hamster prion protein PrP(29-231)
Brain Duchen 116 555 1993 10.1093/brain/116.3.555 Dementia associated with a 216 base pair insertion in the prion protein gene
Annu. Rev. Biochem Englund 62 121 1993 10.1146/annurev.bi.62.070193.001005 The structure and biosynthesis of glycosyl phosphatidylinositol protein anchors
EMBO J Fischer 15 1255 1996 10.1002/j.1460-2075.1996.tb00467.x Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie
Nat. Med Gabizon 2 59 1996 10.1038/nm0196-59 Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease
Biochemistry Harris 32 1009 1993 10.1021/bi00055a003 Processing of a cellular prion protein
Curr. Opin. Struct. Biol Harrison 7 53 1997 10.1016/S0959-440X(97)80007-3 The prion folding problem
Science Hegde 279 827 1998 10.1126/science.279.5352.827 A transmembrane form of the prion protein in neurodegenerative disease
Science Hsiao 250 1587 1990 10.1126/science.1980379 Spontaneous neurodegeneration in transgenic mice with mutant prion protein
Proc. Natl. Acad. Sci. USA Hsiao 91 9126 1994 10.1073/pnas.91.19.9126 Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein
Am. J. Path Kitamoto 140 1285 1992 Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt-Jakob disease
Mol. Brain Res Krasemann 34 173 1995 10.1016/0169-328X(95)00175-R Prion disease associated with a novel nine octapeptide repeat insertion in the PRNP gene
J. Biol. Chem Lehmann 270 24589 1995 10.1074/jbc.270.41.24589 A mutant prion protein displays an aberrant membrane association when expressed in cultured cells
J. Biol. Chem Lehmann 271 1633 1996 10.1074/jbc.271.3.1633 Mutant and infectious prion proteins display common biochemical properties in cultured cells
Proc. Natl. Acad. Sci. USA Lehmann 93 5610 1996 10.1073/pnas.93.11.5610 Two mutant prion proteins expressed in cultured cells acquire biochemical properties reminiscent of the scrapie isoform
J. Biol. Chem Lehmann 272 21479 1997 10.1074/jbc.272.34.21479 Blockade of glycosylation promotes acquisition of scrapie-like properties by the prion protein in cultured cells
Mol. Brain Res Lehmann 52 139 1997 10.1016/S0169-328X(97)00231-3 A wild-type prion protein does not acquire properties of the scrapie isoform when coexpressed with a mutant prion protein in cultured cells
Development Manson 115 117 1992 10.1242/dev.115.1.117 The prion protein gene
Nat. Med Muramoto 3 750 1997 10.1038/nm0797-750 Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an α-helix
Cell Oesch 40 735 1985 10.1016/0092-8674(85)90333-2 A cellular gene encodes scrapie PrP 27-30 protein
Mol. Brain Res Owen 13 155 1992 10.1016/0169-328X(92)90056-H A dementing illness associated with a novel insertion in the prion protein gene
Curr. Opin. Neurol Parchi 8 286 1995 10.1097/00019052-199508000-00007 Human prion diseases
Ann. Neurol Parchi 39 767 1996 10.1002/ana.410390613 Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease
Parchi, P., Gambetti, P., Piccardo, P., and Ghetti, B. (1998). Human prion diseases. In Progress in Pathology 4, N. Kirkham and N.R. Lemoine, eds. (Edinburgh: Churchill Livingstone), pp. 39-77.
J. Biol. Chem Petersen 271 12661 1996 10.1074/jbc.271.21.12661 Effect of the D178N mutation and the codon 129 polymorphism on the metabolism of the prion protein
Am. J. Pathol Piccardo 152 1415 1998 An antibody raised against a conserved sequence of the prion protein recognizes pathological isoforms in human and animal prion diseases, including Creutzfeldt-Jakob disease and bovine spongiform encephalopathy
J. Biol. Chem Priola 273 11980 1998 10.1074/jbc.273.19.11980 Abnormal properties of prion protein with insertional mutations in different cell types
Ann. Neurol Prusiner 35 385 1994 10.1002/ana.410350404 Human prion diseases
Cell Prusiner 93 337 1998 10.1016/S0092-8674(00)81163-0 Prion protein biology
FEBS Lett Riek 413 282 1997 10.1016/S0014-5793(97)00920-4 NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231)
Cell Biol. Int. Rep Rosenberry 15 1133 1991 10.1016/0309-1651(91)90060-V A chemical modification that makes glycoinositol phospholipids resistant to phospholipase C cleavage
Nature Sakaguchi 380 528 1996 10.1038/380528a0 Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene
Cell Scott 73 979 1993 10.1016/0092-8674(93)90275-U Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes
Cell Shmerling 93 203 1998 10.1016/S0092-8674(00)81572-X Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions
J. Biol. Chem Singh 272 28461 1997 10.1074/jbc.272.45.28461 Prion protein aggregation reverted by low temperature in transfected cells carrying a prion protein gene mutation
Cell Stahl 51 229 1987 10.1016/0092-8674(87)90150-4 Scrapie prion protein contains a phosphatidylinositol glycolipid
Biochemistry Stahl 29 5405 1990 10.1021/bi00474a028 Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C
Genes Dev Telling 10 1736 1996 10.1101/gad.10.14.1736 Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice
Cell Westaway 76 117 1994 10.1016/0092-8674(94)90177-5 Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins
Brain Res Wozniak 707 165 1996 10.1016/0006-8993(95)01230-3 MK-801 neurotoxicity in male mice
Young, K., Piccardo, P., Dlouhy, S., Bugiani, O., Tagliavini, F., and Ghetti, B. (1999). The human genetic prion diseases. In Prions: Molecular and Cellular Biology, D.A. Harris, ed. (Wymondham, U.K.: Horizon Scientific Press), 139-175.
*원문 PDF 파일 및 링크정보가 존재하지 않을 경우 KISTI DDS 시스템에서 제공하는 원문복사서비스를 사용할 수 있습니다.
출판사/학술단체 등이 한시적으로 특별한 프로모션 또는 일정기간 경과 후 접근을 허용하여, 출판사/학술단체 등의 사이트에서 이용 가능한 논문
Copyright KISTI. All Rights Reserved.
※ AI-Helper는 부적절한 답변을 할 수 있습니다.