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Article

Hereditary spastic paraplegia: respiratory choke or unactivated substrate?

Cell - 21 Oct 2005

Claypool Steven M, Koehler Carla M

Abstract excerpt

Defects in the mitochondrial AAA protease family member, paraplegin, result in an autosomal recessive form of hereditary spastic paraplegia (HSP). In this issue of Cell, Nolden et al. (2005) report a new molecular mechanism for HSP based on the requirement of paraplegin for the proteolysis of a s...

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Identifiers

PMID: 16239134
DOI: 10.1016/j.cell.2005.10.006

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-16239134-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

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

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Hereditary spastic paraplegia: respiratory choke or unactivated substrate?PMID 16239134Read ↗

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

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

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

Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13).Semantic proximity Read ↗

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

Hereditary spastic paraplegia SPG13 mutation increases structural stability and ATPase activity of human mitochondrial chaperonin.Semantic proximity Read ↗

Linkage between ATP consumption and mechanical unfolding during the protein processing reactions of an AAA+ degradation machine.Semantic proximity Read ↗

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