AFG3L2 (m-AAA protease) degrades SMDT1 that is not assembled in MCU

Stable Identifier
R-HSA-8949659
Type
Reaction [omitted]
Species
Homo sapiens
Compartment
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Free SMDT1 (EMRE) that is not complexed with MICU1 and MICU2 (or MICU3) is degraded by AFG3L2 (m-AAA protease) (Konig et al. 2016). This degradation appears to prevent the formation of MCU complexes that contain MCU and SMDT1 but lack MICU1 and MICU2 (or MICU3) and are therefore unregulated.

Literature References
PubMed ID Title Journal Year
27642048 The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria

König, T, Tröder, SE, Bakka, K, Korwitz, A, Richter-Dennerlein, R, Lampe, PA, Patron, M, Mühlmeister, M, Guerrero-Castillo, S, Brandt, U, Decker, T, Lauria, I, Paggio, A, Rizzuto, R, Rugarli, EI, De Stefani, D, Langer, T

Mol. Cell 2016
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
metalloendopeptidase activity of C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created
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