[Aggregation and toxicity of the proteins with polyQ repeats]. / Agregacja a toksycznosc bialek z powtórzeniami polyQ.
Postepy Biochem
; 51(2): 215-22, 2005.
Article
en Pl
| MEDLINE
| ID: mdl-16209359
ABSTRACT
Expansion of CAG triplet repeats is a cause of at least nine late-onset neurodegenerative disorders. The mutation manifests itself as a long stretch of glutamine repeats. The number of approximately 38 repeats is usually a threshold at which the disease develops and the longer the polyglutamine tract, the earlier the onset of disease. A common feature of these disorders is the presence of protein aggregates which are believed to be formed either by the formation of hydrogen bonds between amide residues or through the action of the enzyme transglutaminase. Mutated proteins may cause neurodegeneration by sequestering vital cellular proteins, inhibiting proteasomal system or by inducing apoptosis. It has been proved that molecular chaperones may block the negative effects of expression of mutated genes and for this reason they are a promising object for various therapeutic research.
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Bases de datos:
MEDLINE
Asunto principal:
Péptidos
/
Chaperonas Moleculares
/
Enfermedades Neurodegenerativas
/
Expansión de Repetición de Trinucleótido
/
Secuencias Repetitivas de Aminoácido
Límite:
Humans
Idioma:
Pl
Revista:
Postepy Biochem
Año:
2005
Tipo del documento:
Article
País de afiliación:
Polonia