Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

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 morphology defects, and axonal degeneration in hereditary spastic paraplegia (HSP). The molecular basis of these defects,...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

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

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

Share to Metalloendopeptidases

Share to Mitochondrial Membranes

Share to Protein Biosynthesis

Share to Ribosomal Proteins

Share to Ribosomes

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 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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

Research trail→

Click a neighbor to travelStep 1 · 12 closest

The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria.PMID 16239145Read ↗

Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia.Semantic proximity Read ↗

Hereditary spastic paraplegia: respiratory choke or unactivated substrate?Semantic proximity Read ↗

Autocatalytic processing of m-AAA protease subunits in mitochondria.Semantic proximity Read ↗

The formation of respiratory chain complexes in mitochondria is under the proteolytic control of the m-AAA protease.Semantic proximity Read ↗

Loss of the Drosophila m-AAA mitochondrial protease paraplegin results in mitochondrial dysfunction, shortened lifespan, and neuronal and muscular degeneration.Semantic proximity Read ↗

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival.Semantic proximity Read ↗

Substrate recognition and processing by a Walker B mutant of the human mitochondrial AAA+ protein CLPX.Semantic proximity Read ↗

Activation of the mitochondrial protein quality control system and actin cytoskeletal alterations in cells harbouring the MELAS mitochondrial DNA mutation.Semantic proximity Read ↗

Role of the novel metallopeptidase Mop112 and saccharolysin for the complete degradation of proteins residing in different subcompartments of mitochondria.Semantic proximity Read ↗

Inactivation of the mitochondrial protease Afg3l2 results in severely diminished respiratory chain activity and widespread defects in mitochondrial gene expressionSemantic proximity Read ↗

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

Lysosome Function and Dysfunction in Hereditary Spastic ParaplegiasSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.