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Article

The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria.

Cell - 21 Oct 2005

Nolden Mark, Ehses Sarah, Koppen Mirko, Bernacchia Andrea, Rugarli Elena I, Langer Thomas

Abstract excerpt

AAA proteases comprise a conserved family of membrane bound ATP-dependent proteases that ensures the quality control of mitochondrial inner-membrane proteins. Inactivation of AAA proteases causes pleiotropic phenotypes in various organisms, including respiratory deficiencies, mitochondrial morpho...

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Share to Adenosine Triphosphatases
Share to ATPases Associated with Diverse Cellular Activities
Share to Metalloendopeptidases

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Mitochondrial Membranes

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Share to Spastic Paraplegia, Hereditary

Source

Identifiers

PMID: 16239145
DOI: 10.1016/j.cell.2005.08.003

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-16239145-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 4 Sept 2026.

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Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases.Semantic proximity Read ↗

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