Prion diseases

被引:19
作者
Collinge, John [1 ]
Palmer, Mark S. [1 ]
机构
[1] St Marys Hosp, Med Sch, Dept Biochem & Mol Genet, London W2 1PG, England
关键词
D O I
10.1016/S0959-437X(05)80156-X
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
There have been remarkably rapid advances in the understanding of prion diseases over the past year. The controversial notion that the transmissible agent may be an abnormal isoform of a host-encoded protein, the prion protein, is now gaining wide acceptance. The conundrum of how a disease can both be inherited as an autosomal dominant condition and also be experimentally transmissible by inoculation is beginning to make sense.
引用
收藏
页码:448 / 454
页数:7
相关论文
共 36 条
  • [11] MUTATION IN CODON-200 OF SCRAPIE AMYLOID PROTEIN GENE IN 2 CLUSTERS OF CREUTZFELDT-JAKOB DISEASE IN SLOVAKIA
    GOLDFARB, LG
    MITROVA, E
    BROWN, P
    TOH, BH
    GAJDUSEK, DC
    [J]. LANCET, 1990, 336 (8713) : 514 - 515
  • [12] MUTATION IN CODON-200 OF SCRAPIE AMYLOID PRECURSOR GENE LINKED TO CREUTZFELDT-JAKOB DISEASE IN SEPHARDIC JEWS OF LIBYAN AND NON-LIBYAN ORIGIN
    GOLDFARB, LG
    KORCZYN, AD
    BROWN, P
    CHAPMAN, J
    GAJDUSEK, DC
    [J]. LANCET, 1990, 336 (8715) : 637 - 638
  • [13] MUTATIONS IN FAMILIAL CREUTZFELDT-JAKOB DISEASE AND GERSTMANN-STRAUSSLER-SCHEINKERS SYNDROME
    GOLDGABER, D
    GOLDFARB, LG
    BROWN, P
    ASHER, DM
    BROWN, WT
    LIN, S
    TEENER, JW
    FEINSTONE, SM
    RUBENSTEIN, R
    KASCSAK, RJ
    BOELLAARD, JW
    GAJDUSEK, DC
    [J]. EXPERIMENTAL NEUROLOGY, 1989, 106 (02) : 204 - 206
  • [14] HARDY J, 1991, LANCET, V337, P1342
  • [15] LINKAGE OF A PRION PROTEIN MISSENSE VARIANT TO GERSTMANN-STRAUSSLER SYNDROME
    HSIAO, K
    BAKER, HF
    CROW, TJ
    POULTER, M
    OWEN, F
    TERWILLIGER, JD
    WESTAWAY, D
    OTT, J
    PRUSINER, SB
    [J]. NATURE, 1989, 338 (6213) : 342 - 345
  • [16] MUTATION OF THE PRION PROTEIN IN LIBYAN JEWS WITH CREUTZFELDT-JAKOB DISEASE
    HSIAO, K
    MEINER, Z
    KAHANA, E
    CASS, C
    KAHANA, I
    AVRAHAMI, D
    SCARLATO, G
    ABRAMSKY, O
    PRUSINER, SB
    GABIZON, R
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1991, 324 (16) : 1091 - 1097
  • [17] SPONTANEOUS NEURODEGENERATION IN TRANSGENIC MICE WITH MUTANT PRION PROTEIN
    HSIAO, KK
    SCOTT, M
    FOSTER, D
    GROTH, DF
    DEARMOND, SJ
    PRUSINER, SB
    [J]. SCIENCE, 1990, 250 (4987) : 1587 - 1590
  • [18] HSLAO KK, 1992, PRION DIS HUMANS ANI
  • [19] PROTEINASE-RESISTANT PRION PROTEIN ACCUMULATION IN SYRIAN-HAMSTER BRAIN CORRELATES WITH REGIONAL PATHOLOGY AND SCRAPIE INFECTIVITY
    JENDROSKA, K
    HEINZEL, FP
    TORCHIA, M
    STOWRING, L
    KRETZSCHMAR, HA
    KON, A
    STERN, A
    PRUSINER, SB
    DEARMOND, SJ
    [J]. NEUROLOGY, 1991, 41 (09) : 1482 - 1490
  • [20] CREUTZFELDT-JAKOB DISEASE - PATTERNS OF WORLDWIDE OCCURRENCE AND THE SIGNIFICANCE OF FAMILIAL AND SPORADIC CLUSTERING
    MASTERS, CL
    HARRIS, JO
    GAJDUSEK, DC
    GIBBS, CJ
    BERNOULLI, C
    ASHER, DM
    [J]. ANNALS OF NEUROLOGY, 1979, 5 (02) : 177 - 188