Processing of SMDT1

Stable Identifier
R-HSA-8949664
Type
Pathway
Species
Homo sapiens
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Proteolytic processing of proSMDT1 (proEMRE) regulates assembly of properly regulated mitochondrial calcium uniporter (MCU) complex (Konig et al. 2016). C2orf47 (MAIP) in a complex with AFG3L2 (m-AAA protease) binds the transit peptide of proSMDT1, promotes cleavage of the transit peptide by mitochondrial processing endopeptidase, and prevents proteolytic destruction of proSMDT1. SMDT1 that is not then incorporated with the regulatory subunits MICU1 and MICU2 (or MICU1 and MICU3 in neurons) into the MCU complex is degraded by AFG3L2, preventing assembly of unregulated MCU. Unprocessed proSMDT1 is proteolyzed by YME1L1.

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
Orthologous Events
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Created