Mitopaths

SMDT1 degradation

Contributor: Saverio Marchi

{"name":"SMDT1 degradation","theorem":{"head":{"_type":"interaction","position":3,"components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3}]},"body":{"_type":"conjunction","components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3},{"_type":"protein","name":"SMDT1","position":1},{"_type":"molecule","name":"ATP","position":1}]}},"steps":[{"head":{"_type":"interaction","position":3,"components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3}]},"body":{"_type":"conjunction","components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3}]}},{"head":{"_type":"interaction","position":3,"components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3},{"_type":"protein","name":"SMDT1","position":1}]},"body":{"_type":"conjunction","components":[{"_type":"interaction","position":3,"components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3}]},{"_type":"protein","name":"SMDT1","position":1}]}},{"head":{"_type":"interaction","position":3,"components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3},{"_type":"protein","name":"SMDT1","position":1},{"_type":"molecule","name":"ATP","position":1}]},"body":{"_type":"conjunction","components":[{"_type":"interaction","position":3,"components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3},{"_type":"protein","name":"SMDT1","position":1}]},{"_type":"molecule","name":"ATP","position":1}]}},{"head":{"_type":"conjunction","components":[{"_type":"interaction","position":3,"components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3}]},{"_type":"molecule","name":"ADP","position":1}]},"body":{"_type":"interaction","position":3,"components":[{"_type":"protein","name":"AFG3L2","position":3},{"_type":"protein","name":"SPG7","position":3},{"_type":"protein","name":"SMDT1","position":1},{"_type":"molecule","name":"ATP","position":1}]}}]}

Theorem

Information

Non-assembled SMDT1 (also known as EMRE), a key component of the Uniporter complex (Uniplex) is degrade by the m-AAA complex in order to ensure a proper assembly of the complex. Cells lacking m-AAA proteases display increased mitochondrial Ca2+ entry due to accumulation of constitutive active MCU-SMDT1 complexes.

References:

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. The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria. Mol Cell. 2016 Oct 6;64(1):148-162. doi: 10.1016/j.molcel.2016.08.020. Epub 2016 Sep 15. PMID: 27642048.

Tsai CW, Wu Y, Pao PC, Phillips CB, Williams C, Miller C, Ranaghan M, Tsai MF. Proteolytic control of the mitochondrial calcium uniporter complex. Proc Natl Acad Sci U S A. 2017 Apr 25;114(17):4388-4393. doi: 10.1073/pnas.1702938114. Epub 2017 Apr 10. PMID: 28396416.

Link to other Databases:

Reactome: https://reactome.org/content/detail/R-HSA-8949659