Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

Human Molecular Genetics

Volumn 11, Issue 9, 2002, Pages 1107-1117

  Ravikumar, Brinda ^a   Duden, Rainer ^a   Rubinsztein, David C ^a  

^a ADDENBROOKE S HOSPITAL   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ALANINE; EPOXOMICIN; GREEN FLUORESCENT PROTEIN; LACTACYSTIN; POLYGLUTAMINE; PROTEASOME; RAPAMYCIN;

ALZHEIMER DISEASE; ANIMAL CELL; ARTICLE; AUTOPHAGY; CELL DEATH; CELL LINE; CONTROLLED STUDY; EXON; HUNTINGTON CHOREA; LYSOSOME; MODEL; NONHUMAN; NUCLEOTIDE REPEAT; OCULOPHARYNGEAL MUSCULAR DYSTROPHY; PARKINSON DISEASE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN CONFORMATION; PROTEIN DEGRADATION; PROTEIN FOLDING;

ANIMALIA;

EID: 0036566266     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/11.9.1107     Document Type: Article

References (34)

1. Protein fates in neurodegenerative proteinopathies: Polyglutamine diseases join the (mis)fold
2. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
3. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
4. Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease
5. Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
6. Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release
7. Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophy
8. Oligomerization of polyalanine expanded PABPN1 facilitates nuclear protein aggregation that is associated with cell death
9. Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death
10. A molecular investigation of true dominance in Huntington's disease
11. Identifies of sequestered proteins in aggregates from cells with induced polyglutamine expression
12. Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation
13. Impairment of the ubiquitin-proteasome system by protein aggregation
14. Separation of cathepsin A-like enzyme and the proteasome: Evidence that lactacystin/beta-lactone is not a specific inhibitor of the proteasome
15. Lactacystin inhibits cathepsin A activity in melanoma cell lines
16. Vacuolar import of proteins and organelles from the cytoplasm
17. Huntingtin expression stimulates endosomal-lysosomal activity, endosomal tubulation and autophagy
18. Autophagy as a regulated pathway of cellular degradation
19. Inhibition of hepatocytic autophagy by adenosine, adenosine analogs and AMP
20. Bafilomycin A1 prevents maturation of autophagic vacuoles by inhibiting fusion between autophagosomes and lysosomes in rat hepatoma cell line, H-4-II-E cells
21. Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease
22. Inhibition of protein synthesis separates autophagic sequestration from the delivery of lysosomal enzymes
23. Dissection of autophagosome biogenesis into distinct nucleation and expansion steps
24. Epoxomicin, a potent and selective proteasome inhibitor, exhibits in vivo anti-inflammatory activity
25. Proteasome inhibition leads to the activation of all members of the heat-shock-factor family
26. Proteasome inhibitors MG132 and lactacystin hyperphosphorylate HSF1 and induce hsp70 and hsp27 expression
27. Sirolimus (rapamycin) for the treatment of steroid-refractory acute graft-versus-host disease1
28. Evolution of immunosuppression and continued importance of acute rejection in renal transplantation
29. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells
30. Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis
31. Mutant protein in Huntington disease is resistant to proteolysis in affected brain
32. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
33. Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation
34. Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice