Substrates for peptidase M16.003: mitochondrial processing peptidase beta-subunit

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature Substrates

Peptide and protein substrates that are thought to be physiologically relevant are indicated by P. Peptide and protein substrates that are thought to be pathologically relevant are indicated by D. Peptide and protein substrates that are not physiologically relevant are indicated by N. Synthetic substrates are indicated by S. Click on the symbol to show only physiological, non-physiological or synthetic substrates, or here to display all substrates. How cleavage sites have been identified are indicated by the following evidence codes: NT = N-terminal sequencing, MS = mass spectroscopy, MU = mutation, CS = consensus sequence, LC = liquid chromatography. To see all annotated cleavages for a protein substrate, click on the UniProt Accession.

Substrate	Uniprot	Residue range	Cleavage Site	Cleavage type	Evidence	P4	P3	P2	P1	P1'	P2'	P3'	P4'	Reference
37S ribosomal protein RSM28, mitochondrial	Q03430	1-361	peptide-His13+Tyr-peptide	P	MS	Ser	Arg	Ala	His	Tyr	Ser	Thr	Ser	Vogtle et al., 2009
54S ribosomal protein L11, mitochondrial	P36521	1-249	peptide-Phe30+Tyr-peptide	P	MS	His	Lys	Arg	Phe	Tyr	Ala	Leu	Ala	Vogtle et al., 2009
54S ribosomal protein L41, mitochondrial	P32387	1-263	peptide-Ser33+Leu-peptide	P	MS	Pro	Ile	Arg	Ser	Leu	Ala	Ser	Val	Vogtle et al., 2009
60 kDa heat shock protein, mitochondrial	P10809	1-573	peptide-Val17+Leu-peptide	P	CS	Val	Ser	Arg	Val	Leu	Ala	Pro	His	Gevaert K et al., 2003
aconitate hydratase, mitochondrial	P16276	1-781	peptide-Cys27+Gln-peptide	P	MS	Ser	Val	Leu	Cys	Gln	Arg	Ala	Lys
aconitate hydratase, mitochondrial	P19414	1-778	peptide-Gly15+Leu-peptide	P	MS	Ile	Val	Arg	Gly	Leu	Ala	Thr	Val	Vogtle et al., 2009
acyl carrier protein, mitochondrial	P32463	1-125	peptide-Phe35+Tyr-peptide	P	MS	Ala	Gln	Arg	Phe	Tyr	Ser	Ala	Asn	Vogtle et al., 2009
adrenodoxin, mitochondrial	P00257	18-53	peptide-Thr50+Thr-Leu-Ser	P	NT	Ala	Thr	Arg	Thr	Leu	Ser	Val	-	Kitada et al., 2007
aFG3-like protein 1	Q920A7	1-789	peptide-Tyr70+Ser-peptide	P	NT	Pro	Arg	Leu	Tyr	Ser	Lys	Pro	Pro	Koppen et al., 2009
AFG3-like protein 2	Q8JZQ2	1-802	peptide-Thr38+Leu-peptide	P	NT	Cys	Leu	Arg	Thr	Leu	Tyr	Gln	Tyr	Koppen et al., 2009
aldehyde dehydrogenase, mitochondrial	P47738	1-29	peptide-Leu18+Leu-peptide	P	NT	Leu	Ser	Arg	Leu	Leu	Ser	Ala	Ala	Kitada et al., 2007
Aspartate aminotransferase, mitochondrial precursor	P05202	1-430	peptide-Ala29+Ser-peptide	P	MS	Ser	Ala	Arg	Ala	Ser	Ser	Trp	Trp	Wang et al., Article
ATP synthase subunit alpha, mitochondrial precursor	P25705	1-553	peptide-Asn35+Phe-peptide	P	MS	Ala	Ala	Arg	Asn	Phe	His	Ala	Ser	Gevaert et al., 2003
ATP synthase subunit b, mitochondrial precursor	P24539	1-256	peptide-Val42+Pro-peptide	P	MS	Pro	His	Leu	Val	Pro	Val	Pro	Pro	Van Damme P et al., 2005
ATP synthase subunit beta, mitochondrial	P23704	1-519	Peptide-Phe40+Ala-Peptide	P	NT	Leu	Ser	Arg	Phe	Ala	Ser	Ser	Ala	Bassham et al., 1994
ATP synthase subunit delta, mitochondrial	Q12165	1-160	peptide-Tyr22+Ala-peptide	P	MS	Lys	Arg	Ser	Tyr	Ala	Glu	Ala	Ala	Vogtle et al., 2009
ATP synthase subunit delta, mitochondrial precursor	P30049	1-168	peptide-Tyr22+Ala-peptide	P	MS	Ala	Arg	Ala	Tyr	Ala	Glu	Ala	Ala	Van Damme P et al., 2005
ATP synthase subunit delta, mitochondrial precursor	Q9D3D9	1-168	peptide-Tyr22+Ala-peptide	P	MS	Ala	Arg	Thr	Tyr	Ala	Glu	Ala	Ala	Wang et al., Article
ATP synthase subunit gamma, mitochondrial	P38077	1-311	peptide-Thr32+Tyr-peptide	P	MS	Pro	Val	Arg	Thr	Tyr	Ala	Thr	Leu	Vogtle et al., 2009
ATP synthase subunit gamma, mitochondrial precursor	P36542	1-298	peptide-Met25+Ala-peptide	P	MS	Val	Arg	Asn	Met	Ala	Thr	Leu	Lys	Van Damme P et al., 2005
ATP synthase subunit O, mitochondrial precursor	P48047	1-213	peptide-Cys15+Phe-peptide	P	MS	Gln	Val	Arg	Cys	Phe	Ser	Thr	Ser	Van Damme P et al., 2005
ATP synthase-coupling factor 6, mitochondrial precursor	P18859	1-108	peptide-Asn24+Ile-peptide	P	MS	Leu	Arg	Arg	Asn	Ile	Gly	Val	Thr	Van Damme P et al., 2005
ATPase inhibitor, mitochondrial	P01097	1-85	peptide-Phe21+Tyr-peptide	P	MS	Ala	Ala	Arg	Phe	Tyr	Ser	Glu	Gly	Vogtle et al., 2009
ATPase-stabilizing factor 9 kDa, mitochondrial	P01098	1-86	peptide-Phe22+Tyr-peptide	P	MS	Ala	Ser	Arg	Phe	Tyr	Ser	Asp	Gly	Vogtle et al., 2009
chaperonin HSP60, mitochondrial	P56281	1-47	peptide-Leu8+Ala-peptide	P	NT	Ala	Val	Arg	Leu	Ala	Gly	Lys	Asp	Bringaud et al., 1995
citrate synthase, mitochondrial	P00890	1-479	peptide-His36+Tyr-peptide	P	MS	Asn	Ala	Arg	His	Tyr	Ser	Ser	Ala	Vogtle et al., 2009
cytochrome b-c1 complex subunit 1, mitochondrial	P07256	1-457	peptide-Ser17+Leu-peptide	P	MS	Phe	Lys	Arg	Ser	Leu	Ala	Thr	Ala	Vogtle et al., 2009
cytochrome b-c1 complex subunit 2, mitochondrial	P07257	1-32	peptide-Arg16+Leu-peptide	P	NT	Ser	Val	Arg	Arg	Leu	Thr	Val	Ser	Kitada et al., 2007
Cytochrome b-c1 complex subunit 6, mitochondrial precursor	P99028	1-89	peptide-Ser13+Gly-peptide	P	MS	Leu	Thr	Gly	Ser	Gly	Asp	Pro	Lys	Wang et al., Article
Cytochrome b-c1 complex subunit 6, mitochondrial precursor	P07919	1-91	peptide-Ser13+Gly-peptide	P	MS	Leu	Thr	Glu	Ser	Gly	Asp	Pro	Glu
cytochrome c oxidase assembly protein COX11, mitochondrial	P19516	1-300	peptide-Ser44+Tyr-peptide	P	MS	Ala	Leu	Arg	Ser	Tyr	Ser	Val	Asn	Vogtle et al., 2009
cytochrome C oxidase polypeptide IV, mitochondrial precursor	P04037	1-155	peptide-Thr17+Leu-peptide	P		Ala	Thr	Arg	Thr	Leu	Cys	Ser	Ser	Hendrick et al., 1989
cytochrome c oxidase subunit 6, mitochondrial	P00427	1-148	peptide-Lys39+Tyr-peptide	P	MS	Gln	Ser	Arg	Lys	Tyr	Ser	Asp	Ala	Vogtle et al., 2009
cytochrome P450 11A1, mitochondrial precursor	P00189	1-520	peptide-Gly39+Ile-peptide	P		Glu	Gly	Ala	Gly	Ile	Ser	Thr	Lys	Kojima et al., 2001
D-lactate dehydrogenase [cytochrome] 2, mitochondrial	P46681	1-530	peptide-Tyr35+Tyr-peptide	P	MS	Arg	Val	Asn	Tyr	Tyr	Ser	Thr	Lys	Vogtle et al., 2009
dehydrogenase/reductase SDR family member 2	Q13268	1-258	peptide-Met23+Ser-peptide	P	NT	Ser	Val	Arg	Met	Ser	Ser	Thr	Gly	Gabrielli et al., 1995
delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial	P07275	1-575	peptide-Ser15+Phe-peptide	P	MS	Phe	Lys	Arg	Ser	Phe	Ser	Gln	Leu	Vogtle et al., 2009
dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial	P12695	1-482	peptide-Cys27+Tyr-peptide	P	MS	Gln	Leu	Arg	Cys	Tyr	Ala	Ser	Tyr	Vogtle et al., 2009
frataxin homolog, mitochondrial precursor	Q07540	1-174	peptide-Tyr20+Met-peptide	P		Gly	Arg	Arg	Tyr	Met	Ile	Ala	Ala	Gordon et al., 2001
frataxin homolog, mitochondrial precursor	Q07540	22-174	peptide-Phe51+Val-peptide	P		Gln	Lys	Arg	Phe	Val	Glu	Ser	Ser	Gordon et al., 2001
frataxin, mitochondrial precursor	Q16595	1-210	peptide-Gly41+Leu-peptide	P		Gly	Arg	Arg	Gly	Leu	Arg	Thr	Asp	Cavadini et al., 2000
frataxin, mitochondrial precursor	Q16595	42-210	peptide-Ala55+Ser-peptide	P		Pro	Arg	Arg	Ala	Ser	Ser	Asn	Gln	Cavadini et al., 2000
Fumarate hydratase, mitochondrial precursor	P07954	1-510	peptide-Met44+Ala-peptide	P	MS	Ala	Ala	Arg	Met	Ala	Ser	Gln	Asn	Van Damme P et al., 2005
glutamate dehydrogenase 1	Q43314	1-411	peptide-Leu17+Leu-peptide	P	NT	Ala	Ala	Arg	Leu	Leu	Gly	Leu	Asp	Grzechowiak et al., 2020
glutaredoxin-2, mitochondrial	P17695	1-143	peptide-Phe29+Leu-peptide	P	MS	Ala	Lys	Arg	Phe	Leu	Ser	Thr	Pro	Vogtle et al., 2009
grpE protein homolog, mitochondrial	P38523	1-228	peptide-Phe43+Tyr-peptide	P	MS	Arg	Met	Arg	Phe	Tyr	Ser	Asp	Glu	Vogtle et al., 2009
heat shock protein 60, mitochondrial	P19882	1-572	peptide-Ala20+Tyr-peptide	P	MS	Leu	Arg	Arg	Ala	Tyr	Ser	Ser	His	Vogtle et al., 2009
heat shock protein SSC1, mitochondrial	P12398	1-32	peptide-Ser16+Phe-peptide	P	NT	Leu	Ser	Ser	Ser	Phe	Arg	Ile	Ala	Kitada et al., 2007
homoisocitrate dehydrogenase, mitochondrial	P40495	1-371	peptide-Gly14+Leu-peptide	P	MS	Ala	Cys	Arg	Gly	Leu	Ala	Ser	Asn	Vogtle et al., 2009
isocitrate dehydrogenase [NAD] subunit 2, mitochondrial	P28241	1-369	peptide-Phe14+Leu-peptide	P	MS	Ser	Arg	Arg	Phe	Leu	Ala	Thr	Val	Vogtle et al., 2009
lysyl-tRNA synthetase, mitochondrial	P32048	1-576	peptide-Pro28+Tyr-peptide	P	MS	Arg	Ser	Arg	Pro	Tyr	Ser	Leu	Ala	Vogtle et al., 2009
malate dehydrogenase, mitochondrial	P17505	2-17	Leu-Ser-Arg-Val-Ala-Lys-Arg-Ala+Phe-Ser-Ser-Thr-Val-Ala-Asn-Pro	P	NT	Ala	Lys	Arg	Ala	Phe	Ser	Ser	Thr	Kitada et al., 2007
malate dehydrogenase, mitochondrial precursor	P08249	1-338	peptide-Ser16+Phe-peptide	P		Leu	Arg	Arg	Ser	Phe	Ser	Thr	Ser	Hendrick et al., 1989
malate dehydrogenase, mitochondrial precursor	P04636	1-338	peptide-Ser16+Phe-peptide	P		Leu	Arg	Arg	Ser	Phe	Ser	Thr	Ser	Hendrick et al., 1989
mitochondrial import inner membrane translocase subunit TIM21	P53220	1-239	peptide-Leu41+Tyr-peptide	P	MS	Arg	Thr	Arg	Leu	Tyr	Ser	Asn	Gly	Vogtle et al., 2009
mitochondrial-processing peptidase subunit alpha	P11914	1-482	peptide-Leu9+Tyr-peptide	P	MS	Val	Gln	Arg	Leu	Tyr	Ser	Asn	Ile	Vogtle et al., 2009
mitochondrial-processing peptidase subunit beta	P10507	1-462	peptide-Leu15+Leu-peptide	P	MS	Thr	Arg	Arg	Leu	Leu	Ser	Thr	Ile	Vogtle et al., 2009
NAD-dependent malic enzyme, mitochondrial	P36013	1-669	peptide-Leu38+Tyr-peptide	P	MS	Gln	Ser	Arg	Leu	Tyr	Ser	Ser	Asn	Vogtle et al., 2009
NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial precursor	P19404	1-249	peptide-Asn24+Leu-peptide	P	MS	His	Val	Arg	Asn	Leu	His	Lys	Thr	Van Damme P et al., 2005
ornithine aminotransferase, mitochondrial	P04181	1-25	peptide-Gly17+Val-peptide	P	NT	Leu	Ser	Arg	Gly	Val	His	Ser	Ser	Kitada et al., 2007
ornithine carbamoyltransferase, mitochondrial precursor	P00481	1-354	peptide-Asn24+Phe-peptide	P		Met	Val	Arg	Asn	Phe	Arg	Tyr	Gly	Sztul etal, 1987
ornithine carbamoyltransferase, mitochondrial precursor	P00480	1-354	peptide-Asn24+Phe-peptide	P		Met	Val	Arg	Asn	Phe	Arg	Cys	Gly	Hendrick et al., 1989
ornithine carbamoyltransferase, mitochondrial precursor	P11725	1-354	peptide-His24+Phe-peptide	P		Val	Val	Arg	His	Phe	Trp	Cys	Gly	Hendrick et al., 1989
paraplegin	Q3ULF4	1-781	peptide-Tyr43+Ala-peptide	P	NT	Arg	Arg	Pro	Tyr	Ala	Ala	Arg	Gly	Koppen et al., 2009
peptidyl-prolyl cis-trans isomerase, mitochondrial precursor	P10255	1-223	peptide-Ala36+Phe-peptide	P		Cys	Ala	Arg	Ala	Phe	Ser	Gln	Thr	Hendrick et al., 1989
peroxiredoxin-5, mitochondrial	P99029	1-210	peptide-Ser41+Phe-peptide	P	NT	Gly	Ala	Arg	Ser	Phe	Ser	Ser	Ser	Sim et al., 2021
phenylalanyl-tRNA synthetase, mitochondrial	P08425	1-469	peptide-Leu16+Tyr-peptide	P	MS	Leu	Tyr	Arg	Leu	Tyr	Ser	Thr	Leu	Vogtle et al., 2009
Phosphate carrier protein, mitochondrial precursor	Q8VEM8	1-357	peptide-Ala45+Ala-peptide	P	MS	Leu	Ala	Ala	Ala	Ala	Val	Glu	Glu	Wang et al., Article
potassium-activated aldehyde dehydrogenase, mitochondrial	P46367	1-519	peptide-Tyr23+Phe-peptide	P	MS	Gln	Leu	Arg	Tyr	Phe	Ser	His	Leu	Vogtle et al., 2009
presequence translocated-associated motor subunit PAM17, mitochondrial	P36147	1-197	peptide-Ser36+Tyr-peptide	P	MS	Pro	Leu	Arg	Ser	Tyr	Ser	Gln	Pro	Vogtle et al., 2009
probable alanine aminotransferase, mitochondrial	P52893	1-592	peptide-Ser77+Leu-peptide	P	MS	His	Arg	Arg	Ser	Leu	Ser	Gly	Gln	Vogtle et al., 2009
protein ATP11, mitochondrial	P32453	1-318	peptide-Phe34+Tyr-peptide	P	MS	Val	Phe	Arg	Phe	Tyr	Ser	Ser	Ser	Vogtle et al., 2009
protein FMP16, mitochondrial	Q12497	1-93	peptide-Ala29+Phe-peptide	P	MS	Thr	Arg	Ala	Ala	Phe	Pro	His	Leu	Vogtle et al., 2009
putative thiosulfate sulfurtransferase FMP31, mitochondrial	Q08742	1-149	peptide-Thr24+Phe-peptide	P	MS	Val	Leu	Arg	Thr	Phe	Thr	Thr	Lys	Vogtle et al., 2009
serine--pyruvate aminotransferase, mitochondrial precursor	P09139	1-414	peptide-Met23+Gly-peptide	P		Val	Arg	Asn	Met	Gly	Ser	His	Gln	Miura et al., 1986
succinate dehydrogenase assembly factor 2, mitochondrial	Q08230	1-162	peptide-Trp35+Phe-peptide	P	MS	Gly	Arg	Arg	Trp	Phe	Ser	Ser	Asp	Vogtle et al., 2009
succinate dehydrogenase B/ribosomal protein S14 polyprotein	Q9S7D3	1-350	peptide-Gly247+Tyr-peptide	P	NT	Gln	Ala	Arg	Gly	Tyr	His	Gly	Val	Oshima et al., 2005
Succinate dehydrogenase cytochrome b560 subunit, mitochondrial precursor	Q99643	1-169	peptide-Pro29+Leu-peptide	P	MS	Asn	Ala	Val	Pro	Leu	Gly	Thr	Thr	Van Damme P et al., 2005
tyrosyl-tRNA synthetase, mitochondrial	P48527	1-492	peptide-Phe36+Tyr-peptide	P	MS	Lys	Ser	Arg	Phe	Tyr	Ser	Gln	Pro	Vogtle et al., 2009
ubiquinol-cytochrome c reductase iron-sulfur subunit, mitochondrial precursor	P07056	1-231	peptide-Ala24+Leu-peptide	P		Ala	Val	Arg	Ala	Leu	Thr	Thr	Ser	Hendrick et al., 1989
uncharacterized mitochondrial membrane protein FMP10	P40098	1-244	peptide-Thr12+Tyr-peptide	P	MS	Gln	Ile	Arg	Thr	Tyr	Thr	Asn	Gly	Vogtle et al., 2009
uncharacterized oxidoreductase YHL021C	P23180	1-465	peptide-Thr22+Tyr-peptide	P	MS	Thr	Pro	Arg	Thr	Tyr	Thr	Ser	Ala	Vogtle et al., 2009
uncharacterized protein YMR157C	Q03798	1-255	peptide-Arg39+Tyr-peptide	P	MS	Leu	Arg	Ala	Arg	Tyr	Ser	Ser	Thr	Vogtle et al., 2009
Uracil-DNA glycosylase	P13051-2	1-80	peptide-Leu29+Cys-peptide	P	NT	Leu	Ser	Arg	Leu	Cys	Gly	Asp	His	Bharati et al., 1998
Uracil-DNA glycosylase	P13051-2	1-80	peptide-Arg75+Leu-peptide	P	NT	Ala	Leu	Leu	Arg	Leu	Ala	Ala	Arg	Bharati et al., 1998
Uracil-DNA glycosylase	P13051-2	1-80	peptide-Ala77+Ala-Arg-Asn	P	NT	Leu	Arg	Leu	Ala	Ala	Arg	Asn	-	Bharati et al., 1998