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Article

Insights in progressive myoclonus epilepsy: HSP70 promotes cystatin B polymerization.

Biochimica et biophysica acta - 1 Dec 2013

Rispoli Ada, Cipollini Elena, Catania Sandra, Di Giaimo Rossella, Pulice Giuseppe, van Houte Stineke, Sparla Francesca, Dal Piaz Fabrizio, Roncarati Davide, Trost Paolo, Melli Marialuisa

Abstract excerpt

Cystatin B (CSTB) is an anti-protease frequently mutated in progressive myoclonus epilepsy (EPM1), a devastating degenerative disease. This work shows that rat CSTB is an unstable protein that undergoes structural changes following the interaction with a chaperone, either prokaryotic or eukaryotic. Both the prokaryotic DnaK and eukaryotic HSP70 promote CSTB polymerization. Denaturated CSTB is polymerized by the...

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Identifiers

PMID: 24063889
DOI: 10.1016/j.bbapap.2013.09.011

Genes

CSTB

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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