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Processing of SMDT1

Summary

Organism

Homo sapiens (human)

Reactome

R-HSA-8949664

PubChem

R-HSA-8949664

Description

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.

Click on a node on the pathway to see its details. Glycoproteins are marked with a glycoprotein icon in their name.

SPV: a JavaScript Signaling Pathway Visualizer, Bioinformatics, 2018

Displaying **all 10** entries
UniProt ID	Protein Name	Gene Symbol	Pathway Viewer
O75439	Mitochondrial-processing peptidase subunit beta	MPPB PMPCB	view
P35232	Prohibitin 1	PHB PHB1	view
Q10713	Mitochondrial-processing peptidase subunit alpha	INPP5E KIAA0123 MPPA PMPCA	view
Q86XE3	Calcium uptake protein 3, mitochondrial	EFHA2 MICU3	view
Q8IYU8	Calcium uptake protein 2, mitochondrial	EFHA1 MICU2	view
Q96TA2	ATP-dependent zinc metalloprotease YME1L1	FTSH1 UNQ1868/PRO4304 YME1L YME1L1	view
Q99623	Prohibitin-2	BAP PHB2 REA	view
Q9H4I9	Essential MCU regulator, mitochondrial	C22orf32 EMRE SMDT1	view
Q9NWR8	Calcium uniporter regulatory subunit MCUb, mitochondrial	CCDC109B MCUB	view
Q9UQ90	Mitochondrial inner membrane m-AAA protease component paraplegin	CAR CMAR PGN SPG7	view

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