Reactome | Processing of SMDT1

Processing of SMDT1

Stable Identifier

R-HSA-8949664

Type

Pathway

Species

Homo sapiens

ReviewStatus

5/5

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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 Bakka, K, König, T, Rizzuto, R, Guerrero-Castillo, S, Korwitz, A, Brandt, U, Langer, T, De Stefani, D, Tröder, SE, Lampe, PA, Rugarli, EI, Decker, T, Richter-Dennerlein, R, Lauria, I, Paggio, A, Mühlmeister, M, Patron, M	Mol. Cell	2016