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Literature summary for 3.4.24.B18 extracted from

  • Wang, S.; Jacquemyn, J.; Murru, S.; Martinelli, P.; Barth, E.; Langer, T.; Niessen, C.; Rugarli, E.
    The mitochondrial m-AAA protease prevents demyelination and hair greying (2016), PLoS Genet., 12, e1006463 .
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.4.24.B18

Organism

Organism UniProt Comment Textmining
Mus musculus Q8JZQ2
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-

Source Tissue

Source Tissue Comment Organism Textmining
oligodendrocyte
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 Mus musculus
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Synonyms

Synonyms Comment Organism
AFG3L2
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 Mus musculus

General Information

General Information Comment Organism
physiological function deletion of subunit AFG3L2 in mature mouse oligodendrocytes provokes early-on mitochondrial fragmentation and swelling. Total ablation of the m-AAA protease, by deleting both Afg3l2 and its paralogue Afg3l1, triggers progressive motor dysfunction and demyelination, owing to rapid oligodendrocyte cell death. The mice show premature hair greying, caused by progressive loss of melanoblasts Mus musculus