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Article

Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase.

Human molecular genetics - 1 Sept 1998

Koutnikova H, Campuzano V, Koenig M

Abstract excerpt

Frataxin is a mitochondrial protein deficient in Friedreich ataxia (FRDA) and which is associated with abnormal intramitochondrial iron handling. We identified the mitochondrial processing peptidase beta (MPPbeta) as a frataxin protein partner using the yeast two-hybrid assay. In in vitro assays, MPPbeta binds frataxin which is cleaved by the reconstituted MPP heterodimer. MPP cleavage of frataxin results in an...

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Identifiers

PMID: 9700204
DOI: 10.1093/hmg/7.9.1485

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase.PMID 9700204Read ↗

In vivo maturation of human frataxin.Semantic proximity Read ↗

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Mitochondrial intermediate peptidase and the yeast frataxin homolog together maintain mitochondrial iron homeostasis in Saccharomyces cerevisiae.Semantic proximity Read ↗

Conformational stability of human frataxin and effect of Friedreich's ataxia-related mutations on protein folding.Semantic proximity Read ↗

Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms.Semantic proximity Read ↗

Analysis of mammalian 20S proteasome biogenesis: the maturation of beta-subunits is an ordered two-step mechanism involving autocatalysis.Semantic proximity Read ↗

The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron.Semantic proximity Read ↗

Highly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells.Semantic proximity Read ↗

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