A68 IS A COMPONENT OF PAIRED HELICAL FILAMENTS OF GERSTMANN-STRAUSSLER-SCHEINKER DISEASE, INDIANA KINDRED

被引：47

作者：

TAGLIAVINI, F

GIACCONE, G

PRELLI, F

VERGA, L

PORRO, M

TROJANOWSKI, JQ

FARLOW, MR

FRANGIONE, B

GHETTI, B

BUGIANI, O

机构：

[1] NYU MED CTR,NEW YORK,NY 10016

[2] UNIV PENN,SCH MED,DEPT PATHOL,PHILADELPHIA,PA 19104

[3] INDIANA UNIV,SCH MED,DEPT NEUROL,INDIANAPOLIS,IN 46202

[4] INDIANA UNIV,SCH MED,DEPT PATHOL,INDIANAPOLIS,IN 46202

来源：

BRAIN RESEARCH | 1993年 / 616卷 / 1-2期

关键词：

GERSTMANN-STRAUSSLER-SCHEINKER DISEASE; ALZHEIMERS DISEASE; NEUROFIBRILLARY TANGLE; PAIRED HELICAL FILAMENT; PROTEIN-TAU; A68;

D O I：

10.1016/0006-8993(93)90226-D

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

Gerstmann-Straussler-Scheinker disease in the Indiana kindred is pathologically characterized by prion protein amyloid deposits and neurofibrillary tangles (NFT) with paired helical filaments (PHF). Using antibodies to various domains of the tau molecule, we investigated the composition of PHF in this family by immunocytochemistry and immunoblot analysis. The results indicate that A68 is a component of NFT in this family as it is in Alzheimer's disease, and suggest that post-translational modifications of tau leading to formation of A68 are not unique to Alzheimer's disease.

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页码：325 / 328

页数：4

相关论文

共 30 条

[1] GERSTMANN-STRAUSSLER SYNDROME - A VARIANT TYPE - AMYLOID PLAQUES AND ALZHEIMERS NEUROFIBRILLARY TANGLES IN CEREBRAL-CORTEX [J].

AMANO, N ;

YAGISHITA, S ;

YOKOI, S ;

ITOH, Y ;

KINOSHITA, J ;

MIZUTANI, T ;

MATSUISHI, T .

ACTA NEUROPATHOLOGICA, 1992, 84 (01) :15-23

[2] THE SWITCH OF TAU-PROTEIN TO AN ALZHEIMER-LIKE STATE INCLUDES THE PHOSPHORYLATION OF 2 SERINE PROLINE MOTIFS UPSTREAM OF THE MICROTUBULE BINDING REGION [J].

BIERNAT, J ;

MANDELKOW, EM ;

SCHROTER, C ;

LICHTENBERGKRAAG, B ;

STEINER, B ;

BERLING, B ;

MEYER, H ;

MERCKEN, M ;

VANDERMEEREN, A ;

GOEDERT, M ;

MANDELKOW, E .

EMBO JOURNAL, 1992, 11 (04) :1593-1597

[3] BETA-PP PARTICIPATES IN PRP-AMYLOID PLAQUES OF GERSTMANN-STRAUSSLER-SCHEINKER DISEAE, INDIANA KINDRED [J].

BUGIANI, O ;

GIACCONE, G ;

VERGA, L ;

POLLO, B ;

FRANGIONE, B ;

FARLOW, MR ;

TAGLIAVINI, F ;

GHETTI, B .

JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 1993, 52 (01) :64-70

[4] LINKAGE OF THE INDIANA KINDRED OF GERSTMANN-STRAUSSLER-SCHEINKER DISEASE TO THE PRION PROTEIN GENE [J].

DLOUHY, SR ;

HSIAO, K ;

FARLOW, MR ;

FOROUD, T ;

CONNEALLY, PM ;

JOHNSON, P ;

PRUSINER, SB ;

HODES, ME ;

GHETTI, B .

NATURE GENETICS, 1992, 1 (01) :64-67

[5]

FARLOW MR, 1991, HDB CLIN NEUROLOGY H, V16, P619

[6]

Gerstmann J., 1936, Z NEUROL, V154, P736, DOI DOI 10.1007/BF02865827

[7] GERSTMANN-STRAUSSLER-SCHEINKER DISEASE .2. NEUROFIBRILLARY TANGLES AND PLAQUES WITH PRP-AMYLOID COEXIST IN AN AFFECTED FAMILY [J].

GHETTI, B ;

TAGLIAVINI, F ;

MASTERS, CL ;

BEYREUTHER, K ;

GIACCONE, G ;

VERGA, L ;

FARLOW, MR ;

CONNEALLY, PM ;

DLOUHY, SR ;

AZZARELLI, B ;

BUGIANI, O .

NEUROLOGY, 1989, 39 (11) :1453-1461

[8] PRION PROTEIN PREAMYLOID AND AMYLOID DEPOSITS IN GERSTMANN-STRAUSSLER-SCHEINKER DISEASE, INDIANA KINDRED [J].

GIACCONE, G ;

VERGA, L ;

BUGIANI, O ;

FRANGIONE, B ;

SERBAN, D ;

PRUSINER, SB ;

FARLOW, MR ;

GHETTI, B ;

TAGLIAVINI, F .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1992, 89 (19) :9349-9353

[9] NEUROFIBRILLARY TANGLES OF THE INDIANA KINDRED OF GERSTMANN-STRAUSSLER-SCHEINKER DISEASE SHARE ANTIGENIC DETERMINANTS WITH THOSE OF ALZHEIMER-DISEASE [J].

GIACCONE, G ;

TAGLIAVINI, F ;

VERGA, L ;

FRANGIONE, B ;

FARLOW, MR ;

BUGIANI, O ;

GHETTI, B .

BRAIN RESEARCH, 1990, 530 (02) :325-329

[10] LOCALIZATION OF THE ALZ-50 EPITOPE IN RECOMBINANT HUMAN MICROTUBULE-ASSOCIATED PROTEIN TAU [J].

GOEDERT, M ;

SPILLANTINI, MG ;

JAKES, R .

NEUROSCIENCE LETTERS, 1991, 126 (02) :149-154