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Article

SPG7 variant escapes phosphorylation-regulated processing by AFG3L2, elevates mitochondrial ROS, and is associated with multiple clinical phenotypes.

Cell reports - 8 May 2014

Almontashiri Naif A M, Chen Hsiao-Huei, Mailloux Ryan J, Tatsuta Takashi, Teng Allen C T, Mahmoud Ahmad B, Ho Tiffany, Stewart Nicolas A S, Rippstein Peter, Harper Mary Ellen, Roberts Robert, Willenborg Christina, Erdmann Jeanette, Pastore Annalisa, McBride Heidi M, Langer Thomas, Stewart Alexandre F R

Abstract excerpt

Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and a...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 24767997
DOI: 10.1016/j.celrep.2014.03.051

Genes

SPG7

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-24767997-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

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

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