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Article

Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia.

Trends in molecular medicine - 1 Jun 2006

Rugarli Elena I, Langer Thomas

Abstract excerpt

Hereditary spastic paraplegia (HSP) is a genetically heterogeneous neurodegenerative disorder that is characterized by progressive and cell-specific axonal degeneration. An autosomal recessive form of the disease is caused by mutations in paraplegin, which is a conserved subunit of the ubiquitous and ATP-dependent m-AAA protease in mitochondria. The m-AAA protease carries out protein quality control in the inner...

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Identifiers

PMID: 16647881
DOI: 10.1016/j.molmed.2006.04.002

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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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