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Article

Dependence of mitochondrial calcium signalling and dynamics on the disaggregase, CLPB.

Nature communications - 21 Mar 2025

D'Angelo Donato, Sánchez-Vázquez Víctor H, Cartes-Saavedra Benjamín, Vecellio Reane Denis, Cupo Ryan R, Delgado de la Herran Hilda, Ghirardo Giorgia, Shorter James, Wevers Ron A, Wortmann Saskia B, Perocchi Fabiana, Rizzuto Rosario, Raffaello Anna, Hajnóczky György

Abstract excerpt

Cells utilize protein disaggregases to avoid abnormal protein aggregation that causes many diseases. Among these, caseinolytic peptidase B protein homolog (CLPB) is localized in the mitochondrial intermembrane space and linked to human disease. Upon CLPB loss, MICU1 and MICU2, regulators of the mitochondrial calcium uniporter complex (mtCU), and OPA1, a main mediator of mitochondrial fusion, become insoluble but...

Read the complete abstract on PubMed

Topics

Humans
Mitochondria
Calcium Signaling
Calcium
Fibroblasts

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Mitochondrial Membrane Transport Proteins

Cation Transport Proteins

Calcium-Binding Proteins

Endopeptidase Clp

GTP Phosphohydrolases

Membrane Potential, Mitochondrial

Mitochondrial Dynamics

Calcium Channels

Animals

Mutation

Mice

Source

Open in PubMed Open full text

Identifiers

PMID: 40118824
DOI: 10.1038/s41467-025-57641-9
PMCID: PMC11928477

Genes

CLPB
OPA1
MICU1
MICU2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 24 Oct 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

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Dependence of mitochondrial calcium signalling and dynamics on the disaggregase, CLPB.PMID 40118824Read ↗

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Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations.Semantic proximity Read ↗

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