Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease

Human Molecular Genetics

Volumn 10, Issue 12, 2001, Pages 1307-1315

  Sittler, Annie ^a   Lurz, Rudi ^a   Lueder, Gerhild ^a   Priller, Josef ^b   Hayer Hartl, Manajit K ^c   Hartl, F Ulrich ^c   Lehrach, Hans ^a   Wanker, Erich E ^a  

^a MAX PLANCK INSTITUTE FOR MOLECULAR GENETICS   (Germany)

^b HUMBOLDT UNIVERSITY   (Germany)

^c MAX PLANCK INSTITUTE OF BIOCHEMISTRY   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

ANSAMYCIN DERIVATIVE; BENZOQUINONE DERIVATIVE; GELDANAMYCIN; GLUTAMINE; HEAT SHOCK PROTEIN 40; HEAT SHOCK PROTEIN 70; HEAT SHOCK PROTEIN 90; HUNTINGTIN;

ANIMAL CELL; ARTICLE; ASSAY; CANCER CELL CULTURE; CELL STRAIN COS1; CONCENTRATION RESPONSE; CONTROLLED STUDY; DEGENERATIVE DISEASE; DRUG DEVELOPMENT; EXON; FILTER; HEAT SHOCK RESPONSE; HUNTINGTON CHOREA; IMMUNOFLUORESCENCE MICROSCOPY; INHIBITION KINETICS; MAMMAL CELL; MODEL; NONHUMAN; NUCLEOTIDE REPEAT; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; PROTEIN INTERACTION;

EID: 0035363805     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/10.12.1307     Document Type: Article

References (38)

1. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
2. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
3. Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein
4. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
5. Protein aggregation in Huntington's and Parkinson's disease: Implications for therapy
6. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
7. Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
8. Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: Implications for Huntington's disease pathology
9. Aggregation of truncated GST-HD exon 1 fusion proteins containing normal range and expanded glutamine repeats
10. Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: Implications for Huntington's disease therapy
11. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
12. Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans
13. Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins
14. Mechanisms of chaperone suppression of polyglutamine disease: Selectivity, synergy and modulation of protein solubility in Drosophila
15. Polyglutamine length-dependent interaction of hsp40 and hsp70 family chaperones with truncated N-terminal huntingtin: Their role in suppression of aggregation and cellular toxicity
16. Preclinical pharmacologic evaluation of geldanamycin as an antitumor agent
17. p185erbB2 binds to GRP94 in vivo. Dissociation of the p185erbB2/GRP94 hetero-complex by benzoquinone ansamycins precedes depletion of p185erbB2
18. Progesterone receptor structure and function altered by geldanamycin, an hsp90-binding agent
19. Heat-shock protein hsp90 governs the activity of pp60v-src kinase
20. Inhibition of heat shock protein HSP90-pp60v-src heteroprotein complex formation by benzoquinone ansamycins: Essential role for stress proteins in oncogenic transformation
21. Identification and structural characterization of the ATP/ADP-binding site in the Hsp90 molecular chaperone
22. Crystal structure of an Hsp90-geldanamycin complex: Targeting of a protein chaperone by an antitumor agent
23. Mutational analysis of Hsp90 function: Interactions with a steroid receptor and a protein kinase
24. Pharmacologic shifting of a balance between protein refolding and degradation mediated by Hsp90
25. Repression of heat shock transcription factor HSF1 activation by HSP90 (HSP90 complex) that forms a stress-sensitive complex with HSF1
26. Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
27. Membrane filter assay for detection of amyloid-like polyglutamine-containing protein aggregates
28. The transcriptional regulation of heat shock genes: A plethora of heat shock factors and regulatory conditions
29. Structural basis for inhibition of the Hsp90 molecular chaperone by the antitumor antibiotics radicicol and geldanamycin
30. DT-Diaphorase expression and tumor cell sensitivity to 17-allylamino, 17-demethoxygeldanamycin, an inhibitor of heat shock protein 90
31. Clinical Genetics, II. Huntington's disease: From the gene to pathophysiology
32. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
33. Structure, localization and transcriptional properties of two classes of retinoic acid receptor alpha fusion proteins in acute promyelocytic leukemia (APL): Structural similarities with a new family of oncoproteins
34. An analysis of 5′-noncoding sequences from 699 vertebrate messenger RNAs
35. Regulation of the heat-shock protein 70 reaction cycle by the mammalian DnaJ homolog, Hsp40
36. High efficiency gene transfer into mammalian cells
37. SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates
38. Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation