BioPAX pathway converted from "MPP hydrolyzes presequence of matrix precursors" in the Reactome database.LEFT-TO-RIGHT3.4.24MPP hydrolyzes presequence of matrix precursorsThe alpha subunit (MAS2, Alpha-MPP) of the mitochondrial processing peptidase (MPP) binds presequences of mitochondrial precursors and the beta subunit (MAS1, Beta-MPP) cleaves the presequence (Yang et al. 1991, Geli 1993, Luciano et al. 1997, Luciano et al. 1998, Taylor et al. 2001, Dvorakova-Hola et al. 2010). After cleavage, proteins destined for the matrix are drawn into the matrix by ATP-dependent interaction with mtHSP70 (SSC1) of the PAM complex.Authored: May, B, 2011-04-27Reviewed: D'Eustachio, P, 2011-11-01Reviewed: Endo, T, 2012-02-12Edited: May, B, 2011-04-27TIM23 PAM:Precursor CargoReactome DB_ID: 1268012mitochondrial inner membraneGENE ONTOLOGYGO:0005743Converted from EntitySet in ReactomePrecursor Cargo of TIM23 PAMPrecursors Imported to the Matrix by TIM23 PAM ComplexReactome DB_ID: 1268013YFH1YFH1 precursorFrataxin homolog, mitochondrial component recommendedName: Frataxin homolog intermediate form /componentFRDA_YEASTReactome DB_ID: 1267986UniProt:Q07540 YFH1YFH1YDL120WFUNCTION Promotes the biosynthesis of heme as well as the assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. Plays a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+). Can store large amounts of the metal in the form of a ferrihydrite mineral by oligomerization. May be involved in regulation of the mitochondrial electron transport chain.SUBUNIT Monomer; increments in mitochondrial iron uptake induce stepwise assembly of species ranging from trimers to 24-mers. Interacts with ISU1. Interacts with YHB1, SDH1, SDH2, AIM45 and CIR1.PTM Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to mature size protein.MISCELLANEOUS Present with 1560 molecules/cell in log phase SD medium.SIMILARITY Belongs to the frataxin family.Saccharomyces cerevisiaeNCBI Taxonomy4932UniProtQ075401EQUAL174EQUALReactome Database ID Release 731267986Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1267986ReactomeR-SCE-12679861Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1267986.1Reactomehttp://www.reactome.orgJAC1J-type co-chaperone JAC1, mitochondrialJAC1_YEASTJAC1Reactome DB_ID: 1267956UniProt:P53193 JAC1JAC1SEO2YGL018CFUNCTION Co-chaperone required for the assembly of iron-sulfur (Fe/S) clusters in mitochondria. Stimulates the ATPase activity of its specialized Hsp70 chaperone partner SSQ1. Binds to the substrate protein ISU1 and targets it to SSQ1. May function together with SSQ1 in the dislocation of the Fe/S cluster from ISU1 and its insertion into apoproteins.SUBUNIT Interacts with the Fe/S cluster assembly protein ISU1.MISCELLANEOUS Present with 2010 molecules/cell in log phase SD medium.SIMILARITY Belongs to the HscB family.UniProtP5319311EQUAL184EQUALReactome Database ID Release 731267956Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1267956ReactomeR-SCE-12679561Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1267956.1HSP60HSP60 precursorHeat shock protein 60, mitochondrialHSP60_YEASTReactome DB_ID: 1267967UniProt:P19882 HSP60HSP60MIF4YLR259CL8479.10FUNCTION May participate in assembly and/or disassembly of proteins imported into the mitochondrion. HSP60 are ATPases and have affinity for unfolded proteins.SIMILARITY Belongs to the chaperonin (HSP60) family.UniProtP198821EQUAL572EQUALReactome Database ID Release 731267967Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1267967ReactomeR-SCE-12679671Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1267967.1Reactome Database ID Release 731268013Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1268013ReactomeR-SCE-12680131Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1268013.11TIM23 ComplexReactome DB_ID: 1252229TIM23Mitochondrial import inner membrane translocase subunit TIM23TIM23_YEASTTIM23Reactome DB_ID: 1252061UniProt:P32897 TIM23TIM23MAS6MIM23MPI3YNR017WN3180FUNCTION Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane.SUBUNIT Component of the TIM23 complex, at least composed of TIM23, TIM17, TIM50 and TIM21. The complex interacts with the TIM44 component of the PAM complex.MISCELLANEOUS Present with 1440 molecules/cell in log phase SD medium.SIMILARITY Belongs to the Tim17/Tim22/Tim23 family.UniProtP328971EQUAL222EQUALReactome Database ID Release 731252061Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252061ReactomeR-SCE-12520611Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252061.11TIM21Mitochondrial import inner membrane translocase subunit TIM21TIM21_YEASTTIM21Reactome DB_ID: 1252108UniProt:P53220 TIM21TIM21YGR033CFUNCTION Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. Required to keep the TOM and the TIM23 complexes in close contact. At some point, it is released from the TOM23 complex to allow protein translocation into the mitochondrial matrix. In the complex, it acts as an antagonist of TIM50 by reducing preprotein accumulation at the TOM23 complex and promotes dissociation of the PAM complex from the TIM23 complex.SUBUNIT Component of the TIM23 complex, at least composed of TIM23, TIM17, TIM50 and TIM21. Interacts directly with TIM23. Interacts with TOM22 component of the TOM complex. Released from the TOM23 complex by the PAM complex, leading to protein translocation into the mitochondrial matrix.MISCELLANEOUS Present with 3770 molecules/cell in log phase SD medium.SIMILARITY Belongs to the TIM21 family.UniProtP5322071EQUAL239EQUALReactome Database ID Release 731252108Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252108ReactomeR-SCE-12521081Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252108.11TIM17Mitochondrial import inner membrane translocase subunit TIM17TIM17_YEASTTIM17Reactome DB_ID: 1252059UniProt:P39515 TIM17TIM17MIM17MPI2SMS1YJL143WJ0648FUNCTION Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane.SUBUNIT Component of the TIM23 complex, at least composed of TIM23, TIM17, TIM50 and TIM21 (PubMed:15797382, PubMed:27265872). The complex interacts with the TIM44 component of the PAM complex (PubMed:15797382).PTM The disulfide bond is required for stabilization of the TIM23 complex.MISCELLANEOUS Present with 1210 molecules/cell in log phase SD medium.SIMILARITY Belongs to the Tim17/Tim22/Tim23 family.UniProtP395151EQUAL158EQUALReactome Database ID Release 731252059Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252059ReactomeR-SCE-12520591Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252059.11TIM50Mitochondrial import inner membrane translocase subunit TIM50TIM50_YEASTTIM50Reactome DB_ID: 1252077UniProt:Q02776 TIM50TIM50YPL063WFUNCTION Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. Required to direct preproteins in transit and direct them to the channel protein TIM23, and possibly facilitates transfer of the translocating proteins from the TOM complex to the TIM23 complex. Does not act as a structural subunit of the TIM23 complex but plays a dynamic role in protein import. Has no phosphatase activity.SUBUNIT Component of the TIM23 complex, at least composed of TIM23, TIM17, TIM50 and TIM21. Interacts directly with TIM23. Interacts with preproteins in transit.DOMAIN The mitochondrial intermembrane region is required for generation but not stabilization of the TIM23 complex.DOMAIN The FCP1 homology domain does not contain the canonical D-x-D-x-[TV] active site, suggesting that it probably does not display phosphatase activity.MISCELLANEOUS Present with 2400 molecules/cell in log phase SD medium.SIMILARITY Belongs to the TIM50 family.UniProtQ0277644EQUAL476EQUALReactome Database ID Release 731252077Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252077ReactomeR-SCE-12520771Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252077.11PAM ComplexReactome DB_ID: 1252243TIM44Mitochondrial import inner membrane translocase subunit TIM44TIM44_YEASTTIM44Reactome DB_ID: 1252195UniProt:Q01852 TIM44TIM44ISP45MIM44MPI1YIL022WFUNCTION Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. Recruits mitochondrial HSP70 and its co-chaperone (MGE1) to drive protein translocation into the matrix using ATP as an energy source.SUBUNIT Component of the PAM complex, at least composed of SSC1 (mtHsp70), MGE1, TIM44, PAM16/TIM16, PAM17 and PAM18/TIM14. Forms part of the receptor complex that consists of at least 3 different proteins (TIM17, TIM23, TIM44).SIMILARITY Belongs to the Tim44 family.UniProtQ018521EQUAL431EQUALReactome Database ID Release 731252195Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252195ReactomeR-SCE-12521951Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252195.11PAM16Mitochondrial import inner membrane translocase subunit TIM16TIM16_YEASTPAM16Reactome DB_ID: 1252044UniProt:P42949 PAM16PAM16TIM16YJL104WJ0822FUNCTION Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. In the complex, it is required to regulate activity of mtHSP70 (SSC1) via its interaction with PAM18/TIM14. May act by positioning PAM18/TIM14 in juxtaposition to mtHSP70 at the translocon to maximize ATPase stimulation.SUBUNIT Homodimer and heterodimer with PAM18. Homodimerization may not be relevant in vivo, while heterodimerization is essential for activity regulation of mtHSP70. Component of the PAM complex, at least composed of mtHsp70, MGE1, TIM44, PAM16, PAM17 and PAM18. Interacts with MDJ2.DOMAIN The J-like region, although related to the J domain does not stimulate ATPase activity of mtHSP70. It nevertheless mediates the heterodimerization with the J domain of PAM18 and is therefore essential for PAM complex function.MISCELLANEOUS Present with 3180 molecules/cell in log phase SD medium.SIMILARITY Belongs to the TIM16/PAM16 family.UniProtP429491EQUAL149EQUALReactome Database ID Release 731252044Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252044ReactomeR-SCE-12520441Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252044.11PAM17Presequence translocated-associated motor subunit PAM17, mitochondrialPAM17_YEASTPAM17Reactome DB_ID: 1252063UniProt:P36147 PAM17PAM17FMP18YKR065CFUNCTION Component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. In the complex, it is required to organize PAM16-PAM18 (TIM16-TIM14) heterodimer.SUBUNIT Component of the PAM complex, at least composed of mtHsp70, MGE1, TIM44, PAM16, PAM17 and PAM18/TIM14.MISCELLANEOUS Present with 3000 molecules/cell in log phase SD medium.SIMILARITY Belongs to the PAM17 family.UniProtP3614715EQUAL197EQUALReactome Database ID Release 731252063Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252063ReactomeR-SCE-12520631Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252063.11PAM18Mitochondrial import inner membrane translocase subunit TIM14TIM14_YEASTPAM18Reactome DB_ID: 1252176UniProt:Q07914 PAM18PAM18TIM14YLR008CFUNCTION Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. In the complex, it is required to stimulate activity of mtHSP70 (SSC1).SUBUNIT Homodimer and heterodimer with PAM16/TIM16. Homodimerization may not be relevant in vivo, while heterodimerization is essential for activity regulation of mtHSP70. Component of the PAM complex, at least composed of mtHsp70, MGE1, TIM44, PAM16, PAM17 and PAM18/TIM14. Interacts directly with mtHsp70. Interacts directly with TIM17 subunit of the TIM23 complex.DOMAIN The J domain is essential for co-chaperone activity and mediates the heterodimerization with the J-like domain of PAM16.SIMILARITY Belongs to the TIM14 family.UniProtQ079141EQUAL168EQUALReactome Database ID Release 731252176Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252176ReactomeR-SCE-12521761Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252176.11MGE1GrpE protein homolog, mitochondrialGRPE_YEASTMGE1Reactome DB_ID: 1252101UniProt:P38523 MGE1MGE1GRPEGRPE1YGE1YOR232WO5099FUNCTION Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. Seems to control the nucleotide-dependent binding of SSC1 to substrate proteins and the association of SSC1 with TIM44.SUBUNIT Component of the PAM complex, at least composed of SSC1 (mtHsp70), MGE1, TIM44, PAM16/TIM16, PAM17 and PAM18/TIM14. Interacts with SSQ1.PTM The N-terminus is blocked.SIMILARITY Belongs to the GrpE family.UniProtP385231EQUAL228EQUALReactome Database ID Release 731252101Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252101ReactomeR-SCE-12521011Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252101.11SSC1Heat shock protein SSC1, mitochondrialHSP77_YEASXSSC1Reactome DB_ID: 2457651UniProt:P0CS91 SSC1SSC1ENS1FUNCTION Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. Constitutes the ATP-driven core of the motor and binds the precursor preprotein. Required for the import of the processed frataxin homolog YFH1 into the mitochondrion (By similarity).FUNCTION Acts as a non-catalytic component of endonuclease SceI (endo.SceI), which cleaves specifically at multiple sites on mitochondrial DNA and produces double-stranded breaks. SSC1 confers broader sequence specificity, greater stability, and higher activity on the catalytic subunit.SUBUNIT Component of the PAM complex, at least composed of SSC1 (mtHsp70), MGE1, TIM44, PAM16/TIM16, PAM17 and PAM18/TIM14. In the complex, SSC1 interacts directly with PAM18 and TIM44. Interacts with NAP1 (By similarity). Component of endonuclease SceI (endo.SceI), which is a heterodimer of ENS2 and SSC1.SIMILARITY Belongs to the heat shock protein 70 family.CAUTION S.cerevisiae displays strain polymorphism with regard to Endo.SceI endouclease activity. This is due to the mitochondrion-encoded, catalytic subunit ENS2, which exhibits strain polymorphism. It can be either present as continuous ORF in the mitochondrial genome (e.g. strain IAM 4274), present but disrupted by the insertion of GC clusters (e.g. strain D273-10B/A), or completely absent in the mitochondrial genome (e.g. strain S288c).UniProtP0CS9124EQUAL655EQUALReactome Database ID Release 732457651Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=2457651ReactomeR-SCE-24576511Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-2457651.11Reactome Database ID Release 731252243Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252243ReactomeR-SCE-12522431Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252243.11Reactome Database ID Release 731252229Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1252229ReactomeR-SCE-12522291Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1252229.11Reactome Database ID Release 731268012Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1268012ReactomeR-SCE-12680121Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1268012.1TIM23 PAM:CargoReactome DB_ID: 1268008Converted from EntitySet in ReactomeCargo of TIM23 PAMProteins Imported to the Matrix by TIM23 PAM ComplexReactome DB_ID: 1268001YFH1Frataxin homolog, mitochondrial component recommendedName: Frataxin homolog intermediate form /componentFRDA_YEASTYFH1Reactome DB_ID: 126798322EQUAL174EQUALReactome Database ID Release 731267983Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1267983ReactomeR-SCE-12679831Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1267983.1HSP60Heat shock protein 60, mitochondrialHSP60_YEASTHSP60Reactome DB_ID: 126798226EQUAL572EQUALReactome Database ID Release 731267982Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1267982ReactomeR-SCE-12679821Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1267982.1Reactome Database ID Release 731268001Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1268001ReactomeR-SCE-12680011Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1268001.111Reactome Database ID Release 731268008Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1268008ReactomeR-SCE-12680081Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1268008.1ACTIVATIONactiveUnit: #Protein18Mitochondrial processing peptidaseReactome DB_ID: 1268009mitochondrial matrixGENE ONTOLOGYGO:0005759MAS1Mitochondrial-processing peptidase subunit betaMPPB_YEASTMAS1Reactome DB_ID: 1267976UniProt:P10507 MAS1MAS1MIF1YLR163CL9632.10FUNCTION Catalytic subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins (PubMed:2007593, PubMed:9299349, PubMed:9654444). Preferentially, cleaves after an arginine at position P2 (By similarity).ACTIVITY REGULATION Binding to MAS2 is required for catalytic activity (PubMed:9654444, PubMed:9299349). Inhibited by high levels (> 1uM) of zinc (PubMed:9654444). Inhibited by metal chelators ethylenediaminetetraacetic acid (EDTA) and O-phenanthroline (PubMed:9654444).SUBUNIT Heterodimer of MAS2 (alpha) and MAS1 (beta) subunits, forming the mitochondrial processing protease (MPP) in which MAS2 is involved in substrate recognition and binding and MAS1 is the catalytic subunit.MISCELLANEOUS Present with 1360 molecules/cell in log phase SD medium.SIMILARITY Belongs to the peptidase M16 family.UniProtP1050721EQUAL462EQUALReactome Database ID Release 731267976Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1267976ReactomeR-SCE-12679761Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1267976.11MAS2Mitochondrial-processing peptidase subunit alphaMPPA_YEASTMAS2Reactome DB_ID: 1267987UniProt:P11914 MAS2MAS2MIF2YHR024CFUNCTION Substrate recognition and binding subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins.SUBUNIT Heterodimer of MAS2 (alpha) and MAS1 (beta) subunits, forming the mitochondrial processing protease (MPP) in which MAS2 is involved in substrate recognition and binding and MAS1 is the catalytic subunit.MISCELLANEOUS Present with 31400 molecules/cell in log phase SD medium.SIMILARITY Belongs to the peptidase M16 family.CAUTION Was originally thought to be the catalytic subunit (PubMed:3061797). The low processing activity which was previously observed with alpha-MPP which has been purified from a mitochondrial extract is most likely due to contamination by the beta-subunit. Does not seem to have a protease activity as it lack the zinc-binding site (PubMed:9299349).UniProtP1191414EQUAL482EQUALReactome Database ID Release 731267987Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1267987ReactomeR-SCE-12679871Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1267987.11Reactome Database ID Release 731268009Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1268009ReactomeR-SCE-12680091Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1268009.1GENE ONTOLOGYGO:0004222gene ontology term for cellular functionMIMI:0355Same Catalyst ActivityReactome Database ID Release 731268016Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1268016Reactome Database ID Release 731268017Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=1268017ReactomeR-SCE-12680171Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-SCE-1268017.111344168Pubmed2001Modular structure of the TIM23 preprotein translocase of mitochondriaMilisav, IMoro, FNeupert, WBrunner, MJ Biol Chem 276:25856-619299349Pubmed1997Functional cooperation of the mitochondrial processing peptidase subunitsLuciano, PGeoffroy, SBrandt, AHernandez, JFGéli, VJ Mol Biol 272:213-2520053354Pubmed2010Glycine-rich loop of mitochondrial processing peptidase alpha-subunit is responsible for substrate recognition by a mechanism analogous to mitochondrial receptor Tom20Dvoráková-Holá, KMatusková, AKubala, MOtyepka, MKucera, TVecer, JHerman, PParkhomenko, NKutejova, EJanata, JJ Mol Biol 396:1197-2102007593Pubmed1991The MAS-encoded processing protease of yeast mitochondria. Interaction of the purified enzyme with signal peptides and a purified precursor proteinYang, MJGeli, VOppliger, WSuda, KJames, PSchatz, GJ Biol Chem 266:6416-2311470436Pubmed2001Crystal structures of mitochondrial processing peptidase reveal the mode for specific cleavage of import signal sequencesTaylor, ABSmith, BSKitada, SKojima, KMiyaura, HOtwinowski, ZIto, ADeisenhofer, JStructure 9:615-259654444Pubmed1998The mitochondrial processing peptidase behaves as a zinc-metallopeptidaseLuciano, PTokatlidis, KChambre, IGermanique, JCGéli, VJ Mol Biol 280:193-98327505Pubmed1993Functional reconstitution in Escherichia coli of the yeast mitochondrial matrix peptidase from its two inactive subunitsGéli, VProc Natl Acad Sci U S A 90:6247-51