DAR - Bologna Biocomputing Group

Search results (Reactome ID: R-HSA-1268020)

DiseaseID	Gene	UniProt	EC numbers	Reactomes	Expanded Reactomes	Reactome roots	Reactions
MONDO:0007803	COQ2	Q96H96	2.5.1.39	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-2142789 R-HSA-2142789 Reactome Name: Ubiquinol biosynthesis See in Reactome	R-HSA-196854 R-HSA-196854 Reactome Name: Metabolism of vitamins and cofactors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-8978934 R-HSA-8978934 Reactome Name: Metabolism of cofactors See in Reactome	Metabolism Protein localization	R-HSA-1299476 ( R-HSA-1268020 ) R-HSA-1299476 Reaction Name: MPP cleaves targeting peptide (presequence) of inner membrane precursors See in Reactome R-HSA-1299482 ( R-HSA-1268020 ) R-HSA-1299482 Reaction Name: TIMM23 SORT inserts proteins into inner membrane See in Reactome R-HSA-1299487 ( R-HSA-1268020 ) R-HSA-1299487 Reaction Name: Precursor proteins enter TIMM23 SORT See in Reactome R-HSA-2162192 ( R-HSA-2142789 ) R-HSA-2162192 Reaction Name: PHB and all-E-10PrP2 are combined into DHB by COQ2 See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome
MONDO:0008922	SLC25A4	P12235	-	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-83936 R-HSA-83936 Reactome Name: Transport of nucleosides and free purine and pyrimidine bases across the plasma membrane See in Reactome	R-HSA-425407 R-HSA-425407 Reactome Name: SLC-mediated transmembrane transport See in Reactome R-HSA-425397 R-HSA-425397 Reactome Name: Transport of vitamins, nucleosides, and related molecules See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-382551 R-HSA-382551 Reactome Name: Transport of small molecules See in Reactome	Protein localization Transport of small molecules	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-1299481 ( R-HSA-1268020 ) R-HSA-1299481 Reaction Name: TIMM9:TIMM10 binds hydrophobic proteins See in Reactome R-HSA-1307803 ( R-HSA-1268020 ) R-HSA-1307803 Reaction Name: TIMM22 inserts proteins into inner membrane See in Reactome R-HSA-1955380 ( R-HSA-1268020 ) R-HSA-1955380 Reaction Name: TIMM9:TIMM10 transfers proteins to TIMM22 See in Reactome R-HSA-5250209 ( R-HSA-83936 ) R-HSA-5250209 Reaction Name: ARL2:GTP:ARL2BP binds SLC25A4 See in Reactome R-HSA-5672027 ( R-HSA-83936 ) R-HSA-5672027 Reaction Name: ARL2:GTP:ARL2BP:SLC25A4 dimer exchanges ATP for ADP across the mitochondrial inner membrane See in Reactome
MONDO:0009245	FXN	Q16595	1.16.3.1	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-1362409 R-HSA-1362409 Reactome Name: Mitochondrial iron-sulfur cluster biogenesis See in Reactome	R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-1299475 ( R-HSA-1268020 ) R-HSA-1299475 Reaction Name: TIMM23 PAM translocates proteins from the mitochondrial intermembrane space to the mitochondrial matrix See in Reactome R-HSA-1299478 ( R-HSA-1268020 ) R-HSA-1299478 Reaction Name: MPP cleaves targeting peptide (presequence) of matrix precursors See in Reactome R-HSA-1299480 ( R-HSA-1268020 ) R-HSA-1299480 Reaction Name: Precursor proteins enter TIMM23 PAM See in Reactome R-HSA-1362408 ( R-HSA-1362409 ) R-HSA-1362408 Reaction Name: FXN:NFS1:ISD11:ISCU assembles 2Fe-2S iron-sulfur cluster See in Reactome R-HSA-1362416 ( R-HSA-1362409 ) R-HSA-1362416 Reaction Name: Frataxin binds iron See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome
MONDO:0009505	LDHD	Q86WU2	1.1.2.4	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome	R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Protein localization	R-HSA-1299476 ( R-HSA-1268020 ) R-HSA-1299476 Reaction Name: MPP cleaves targeting peptide (presequence) of inner membrane precursors See in Reactome R-HSA-1299482 ( R-HSA-1268020 ) R-HSA-1299482 Reaction Name: TIMM23 SORT inserts proteins into inner membrane See in Reactome R-HSA-1299487 ( R-HSA-1268020 ) R-HSA-1299487 Reaction Name: Precursor proteins enter TIMM23 SORT See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome
MONDO:0010543	TAFAZZIN	Q16635	2.3.1.-	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-1482798 R-HSA-1482798 Reactome Name: Acyl chain remodeling of CL See in Reactome	R-HSA-556833 R-HSA-556833 Reactome Name: Metabolism of lipids See in Reactome R-HSA-1483206 R-HSA-1483206 Reactome Name: Glycerophospholipid biosynthesis See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-1483257 R-HSA-1483257 Reactome Name: Phospholipid metabolism See in Reactome	Metabolism Protein localization	R-HSA-1299481 ( R-HSA-1268020 ) R-HSA-1299481 Reaction Name: TIMM9:TIMM10 binds hydrophobic proteins See in Reactome R-HSA-1482781 ( R-HSA-1482798 ) R-HSA-1482781 Reaction Name: MLCL and PC are converted to CL and 1-acyl LPC by TAZ (IM) (Reversible) See in Reactome R-HSA-1482794 ( R-HSA-1482798 ) R-HSA-1482794 Reaction Name: CL and 1-acyl LPC are converted to MLCL and PC by TAZ (IM) (Reversible) See in Reactome R-HSA-1482850 ( R-HSA-1482798 ) R-HSA-1482850 Reaction Name: MLCL and PE are converted to CL and 1-acyl LPE by TAZ (IM) (Reversible) See in Reactome R-HSA-1482894 ( R-HSA-1482798 ) R-HSA-1482894 Reaction Name: CL and 1-acyl LPE are converted to MLCL and PE by TAZ (IM) (Reversible) See in Reactome R-HSA-1955380 ( R-HSA-1268020 ) R-HSA-1955380 Reaction Name: TIMM9:TIMM10 transfers proteins to TIMM22 See in Reactome
MONDO:0010703	OTC	P00480	2.1.3.3	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-70635 R-HSA-70635 Reactome Name: Urea cycle See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-1299475 ( R-HSA-1268020 ) R-HSA-1299475 Reaction Name: TIMM23 PAM translocates proteins from the mitochondrial intermembrane space to the mitochondrial matrix See in Reactome R-HSA-1299478 ( R-HSA-1268020 ) R-HSA-1299478 Reaction Name: MPP cleaves targeting peptide (presequence) of matrix precursors See in Reactome R-HSA-1299480 ( R-HSA-1268020 ) R-HSA-1299480 Reaction Name: Precursor proteins enter TIMM23 PAM See in Reactome R-HSA-70560 ( R-HSA-70635 ) R-HSA-70560 Reaction Name: carbamoyl phosphate + ornithine => citrulline + orthophosphate See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome
MONDO:0011532	HSPD1	P10809	5.6.1.7	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-8869496 R-HSA-8869496 Reactome Name: TFAP2A acts as a transcriptional repressor during retinoic acid induced cell differentiation See in Reactome	R-HSA-212436 R-HSA-212436 Reactome Name: Generic Transcription Pathway See in Reactome R-HSA-74160 R-HSA-74160 Reactome Name: Gene expression (Transcription) See in Reactome R-HSA-8864260 R-HSA-8864260 Reactome Name: Transcriptional regulation by the AP-2 (TFAP2) family of transcription factors See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-73857 R-HSA-73857 Reactome Name: RNA Polymerase II Transcription See in Reactome	Gene expression (Transcription) Protein localization	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-1299475 ( R-HSA-1268020 ) R-HSA-1299475 Reaction Name: TIMM23 PAM translocates proteins from the mitochondrial intermembrane space to the mitochondrial matrix See in Reactome R-HSA-1299478 ( R-HSA-1268020 ) R-HSA-1299478 Reaction Name: MPP cleaves targeting peptide (presequence) of matrix precursors See in Reactome R-HSA-1299480 ( R-HSA-1268020 ) R-HSA-1299480 Reaction Name: Precursor proteins enter TIMM23 PAM See in Reactome R-HSA-8869558 ( R-HSA-8869496 ) R-HSA-8869558 Reaction Name: HSPD1 gene expression is repressed by the complex of NPM1 and TFAP2A homodimer See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome
MONDO:0011829	COQ2	Q96H96	2.5.1.39	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-2142789 R-HSA-2142789 Reactome Name: Ubiquinol biosynthesis See in Reactome	R-HSA-196854 R-HSA-196854 Reactome Name: Metabolism of vitamins and cofactors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-8978934 R-HSA-8978934 Reactome Name: Metabolism of cofactors See in Reactome	Metabolism Protein localization	R-HSA-1299476 ( R-HSA-1268020 ) R-HSA-1299476 Reaction Name: MPP cleaves targeting peptide (presequence) of inner membrane precursors See in Reactome R-HSA-1299482 ( R-HSA-1268020 ) R-HSA-1299482 Reaction Name: TIMM23 SORT inserts proteins into inner membrane See in Reactome R-HSA-1299487 ( R-HSA-1268020 ) R-HSA-1299487 Reaction Name: Precursor proteins enter TIMM23 SORT See in Reactome R-HSA-2162192 ( R-HSA-2142789 ) R-HSA-2162192 Reaction Name: PHB and all-E-10PrP2 are combined into DHB by COQ2 See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome
MONDO:0012238	SLC25A4	P12235	-	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-83936 R-HSA-83936 Reactome Name: Transport of nucleosides and free purine and pyrimidine bases across the plasma membrane See in Reactome	R-HSA-425407 R-HSA-425407 Reactome Name: SLC-mediated transmembrane transport See in Reactome R-HSA-425397 R-HSA-425397 Reactome Name: Transport of vitamins, nucleosides, and related molecules See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-382551 R-HSA-382551 Reactome Name: Transport of small molecules See in Reactome	Protein localization Transport of small molecules	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-1299481 ( R-HSA-1268020 ) R-HSA-1299481 Reaction Name: TIMM9:TIMM10 binds hydrophobic proteins See in Reactome R-HSA-1307803 ( R-HSA-1268020 ) R-HSA-1307803 Reaction Name: TIMM22 inserts proteins into inner membrane See in Reactome R-HSA-1955380 ( R-HSA-1268020 ) R-HSA-1955380 Reaction Name: TIMM9:TIMM10 transfers proteins to TIMM22 See in Reactome R-HSA-5250209 ( R-HSA-83936 ) R-HSA-5250209 Reaction Name: ARL2:GTP:ARL2BP binds SLC25A4 See in Reactome R-HSA-5672027 ( R-HSA-83936 ) R-HSA-5672027 Reaction Name: ARL2:GTP:ARL2BP:SLC25A4 dimer exchanges ATP for ADP across the mitochondrial inner membrane See in Reactome
MONDO:0012824	HSPD1	P10809	5.6.1.7	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-8869496 R-HSA-8869496 Reactome Name: TFAP2A acts as a transcriptional repressor during retinoic acid induced cell differentiation See in Reactome	R-HSA-212436 R-HSA-212436 Reactome Name: Generic Transcription Pathway See in Reactome R-HSA-74160 R-HSA-74160 Reactome Name: Gene expression (Transcription) See in Reactome R-HSA-8864260 R-HSA-8864260 Reactome Name: Transcriptional regulation by the AP-2 (TFAP2) family of transcription factors See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-73857 R-HSA-73857 Reactome Name: RNA Polymerase II Transcription See in Reactome	Gene expression (Transcription) Protein localization	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-1299475 ( R-HSA-1268020 ) R-HSA-1299475 Reaction Name: TIMM23 PAM translocates proteins from the mitochondrial intermembrane space to the mitochondrial matrix See in Reactome R-HSA-1299478 ( R-HSA-1268020 ) R-HSA-1299478 Reaction Name: MPP cleaves targeting peptide (presequence) of matrix precursors See in Reactome R-HSA-1299480 ( R-HSA-1268020 ) R-HSA-1299480 Reaction Name: Precursor proteins enter TIMM23 PAM See in Reactome R-HSA-8869558 ( R-HSA-8869496 ) R-HSA-8869558 Reaction Name: HSPD1 gene expression is repressed by the complex of NPM1 and TFAP2A homodimer See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome
MONDO:0013116	GFER	P55789	1.8.3.2	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome	R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Protein localization	R-HSA-1307802 ( R-HSA-1268020 ) R-HSA-1307802 Reaction Name: MIA40:ERV1 (CHCHD4:GFER) oxidizes cysteine residues to cystine disulfide bonds See in Reactome
MONDO:0013802	ACO2	Q99798	4.2.1.3	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-71403 R-HSA-71403 Reactome Name: Citric acid cycle (TCA cycle) See in Reactome	R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-450975 ( R-HSA-71403 ) R-HSA-450975 Reaction Name: isocitrate <=> citrate See in Reactome R-HSA-70971 ( R-HSA-71403 ) R-HSA-70971 Reaction Name: citrate <=> isocitrate See in Reactome
MONDO:0014175	SLC25A4	P12235	-	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-83936 R-HSA-83936 Reactome Name: Transport of nucleosides and free purine and pyrimidine bases across the plasma membrane See in Reactome	R-HSA-425407 R-HSA-425407 Reactome Name: SLC-mediated transmembrane transport See in Reactome R-HSA-425397 R-HSA-425397 Reactome Name: Transport of vitamins, nucleosides, and related molecules See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-382551 R-HSA-382551 Reactome Name: Transport of small molecules See in Reactome	Protein localization Transport of small molecules	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-1299481 ( R-HSA-1268020 ) R-HSA-1299481 Reaction Name: TIMM9:TIMM10 binds hydrophobic proteins See in Reactome R-HSA-1307803 ( R-HSA-1268020 ) R-HSA-1307803 Reaction Name: TIMM22 inserts proteins into inner membrane See in Reactome R-HSA-1955380 ( R-HSA-1268020 ) R-HSA-1955380 Reaction Name: TIMM9:TIMM10 transfers proteins to TIMM22 See in Reactome R-HSA-5250209 ( R-HSA-83936 ) R-HSA-5250209 Reaction Name: ARL2:GTP:ARL2BP binds SLC25A4 See in Reactome R-HSA-5672027 ( R-HSA-83936 ) R-HSA-5672027 Reaction Name: ARL2:GTP:ARL2BP:SLC25A4 dimer exchanges ATP for ADP across the mitochondrial inner membrane See in Reactome
MONDO:0014194	CYC1	P08574	7.1.1.8	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-611105 R-HSA-611105 Reactome Name: Respiratory electron transport See in Reactome	R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-163200 R-HSA-163200 Reactome Name: Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-164651 ( R-HSA-611105 ) R-HSA-164651 Reaction Name: Electron transfer from ubiquinol to cytochrome c of complex III See in Reactome
MONDO:0014571	ACO2	Q99798	4.2.1.3	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-71403 R-HSA-71403 Reactome Name: Citric acid cycle (TCA cycle) See in Reactome	R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-450975 ( R-HSA-71403 ) R-HSA-450975 Reaction Name: isocitrate <=> citrate See in Reactome R-HSA-70971 ( R-HSA-71403 ) R-HSA-70971 Reaction Name: citrate <=> isocitrate See in Reactome
MONDO:0014959	SLC25A4	P12235	-	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-83936 R-HSA-83936 Reactome Name: Transport of nucleosides and free purine and pyrimidine bases across the plasma membrane See in Reactome	R-HSA-425407 R-HSA-425407 Reactome Name: SLC-mediated transmembrane transport See in Reactome R-HSA-425397 R-HSA-425397 Reactome Name: Transport of vitamins, nucleosides, and related molecules See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-382551 R-HSA-382551 Reactome Name: Transport of small molecules See in Reactome	Protein localization Transport of small molecules	R-HSA-1268022 ( R-HSA-1268020 ) R-HSA-1268022 Reaction Name: TOMM40 complex translocates proteins from the cytosol to the mitochondrial intermembrane space See in Reactome R-HSA-1299481 ( R-HSA-1268020 ) R-HSA-1299481 Reaction Name: TIMM9:TIMM10 binds hydrophobic proteins See in Reactome R-HSA-1307803 ( R-HSA-1268020 ) R-HSA-1307803 Reaction Name: TIMM22 inserts proteins into inner membrane See in Reactome R-HSA-1955380 ( R-HSA-1268020 ) R-HSA-1955380 Reaction Name: TIMM9:TIMM10 transfers proteins to TIMM22 See in Reactome R-HSA-5250209 ( R-HSA-83936 ) R-HSA-5250209 Reaction Name: ARL2:GTP:ARL2BP binds SLC25A4 See in Reactome R-HSA-5672027 ( R-HSA-83936 ) R-HSA-5672027 Reaction Name: ARL2:GTP:ARL2BP:SLC25A4 dimer exchanges ATP for ADP across the mitochondrial inner membrane See in Reactome
MONDO:0015470	TAFAZZIN	Q16635	2.3.1.-	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-1482798 R-HSA-1482798 Reactome Name: Acyl chain remodeling of CL See in Reactome	R-HSA-556833 R-HSA-556833 Reactome Name: Metabolism of lipids See in Reactome R-HSA-1483206 R-HSA-1483206 Reactome Name: Glycerophospholipid biosynthesis See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-1483257 R-HSA-1483257 Reactome Name: Phospholipid metabolism See in Reactome	Metabolism Protein localization	R-HSA-1299481 ( R-HSA-1268020 ) R-HSA-1299481 Reaction Name: TIMM9:TIMM10 binds hydrophobic proteins See in Reactome R-HSA-1482781 ( R-HSA-1482798 ) R-HSA-1482781 Reaction Name: MLCL and PC are converted to CL and 1-acyl LPC by TAZ (IM) (Reversible) See in Reactome R-HSA-1482794 ( R-HSA-1482798 ) R-HSA-1482794 Reaction Name: CL and 1-acyl LPC are converted to MLCL and PC by TAZ (IM) (Reversible) See in Reactome R-HSA-1482850 ( R-HSA-1482798 ) R-HSA-1482850 Reaction Name: MLCL and PE are converted to CL and 1-acyl LPE by TAZ (IM) (Reversible) See in Reactome R-HSA-1482894 ( R-HSA-1482798 ) R-HSA-1482894 Reaction Name: CL and 1-acyl LPE are converted to MLCL and PE by TAZ (IM) (Reversible) See in Reactome R-HSA-1955380 ( R-HSA-1268020 ) R-HSA-1955380 Reaction Name: TIMM9:TIMM10 transfers proteins to TIMM22 See in Reactome
MONDO:0020715	COQ2	Q96H96	2.5.1.39	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-2142789 R-HSA-2142789 Reactome Name: Ubiquinol biosynthesis See in Reactome	R-HSA-196854 R-HSA-196854 Reactome Name: Metabolism of vitamins and cofactors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-8978934 R-HSA-8978934 Reactome Name: Metabolism of cofactors See in Reactome	Metabolism Protein localization	R-HSA-1299476 ( R-HSA-1268020 ) R-HSA-1299476 Reaction Name: MPP cleaves targeting peptide (presequence) of inner membrane precursors See in Reactome R-HSA-1299482 ( R-HSA-1268020 ) R-HSA-1299482 Reaction Name: TIMM23 SORT inserts proteins into inner membrane See in Reactome R-HSA-1299487 ( R-HSA-1268020 ) R-HSA-1299487 Reaction Name: Precursor proteins enter TIMM23 SORT See in Reactome R-HSA-2162192 ( R-HSA-2142789 ) R-HSA-2162192 Reaction Name: PHB and all-E-10PrP2 are combined into DHB by COQ2 See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome
MONDO:0054785	PMPCB	O75439	3.4.24.64	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-8949664 R-HSA-8949664 Reactome Name: Processing of SMDT1 See in Reactome	R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome R-HSA-382551 R-HSA-382551 Reactome Name: Transport of small molecules See in Reactome R-HSA-8949215 R-HSA-8949215 Reactome Name: Mitochondrial calcium ion transport See in Reactome	Protein localization Transport of small molecules	R-HSA-1299476 ( R-HSA-1268020 ) R-HSA-1299476 Reaction Name: MPP cleaves targeting peptide (presequence) of inner membrane precursors See in Reactome R-HSA-1299478 ( R-HSA-1268020 ) R-HSA-1299478 Reaction Name: MPP cleaves targeting peptide (presequence) of matrix precursors See in Reactome R-HSA-8949649 ( R-HSA-8949664 ) R-HSA-8949649 Reaction Name: PMPCA:PMPCB cleaves the transit peptide of proSMDT1 (proEMRE) See in Reactome
MONDO:0100340	FXN	Q16595	1.16.3.1	R-HSA-1268020 R-HSA-1268020 Reactome Name: Mitochondrial protein import See in Reactome R-HSA-1362409 R-HSA-1362409 Reactome Name: Mitochondrial iron-sulfur cluster biogenesis See in Reactome	R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-1299475 ( R-HSA-1268020 ) R-HSA-1299475 Reaction Name: TIMM23 PAM translocates proteins from the mitochondrial intermembrane space to the mitochondrial matrix See in Reactome R-HSA-1299478 ( R-HSA-1268020 ) R-HSA-1299478 Reaction Name: MPP cleaves targeting peptide (presequence) of matrix precursors See in Reactome R-HSA-1299480 ( R-HSA-1268020 ) R-HSA-1299480 Reaction Name: Precursor proteins enter TIMM23 PAM See in Reactome R-HSA-1362408 ( R-HSA-1362409 ) R-HSA-1362408 Reaction Name: FXN:NFS1:ISD11:ISCU assembles 2Fe-2S iron-sulfur cluster See in Reactome R-HSA-1362416 ( R-HSA-1362409 ) R-HSA-1362416 Reaction Name: Frataxin binds iron See in Reactome R-HSA-8986181 ( R-HSA-1268020 ) R-HSA-8986181 Reaction Name: PITRM1 proteolyzes mitochondrial targeting peptides (presequences) See in Reactome

Number of enzymes:	11
Number of disease:	20
Number of reactomes:	10
Number of reactions:	27

Search summary (Reactome ID: R-HSA-1268020)

Search results (Reactome ID: R-HSA-1268020)

MONDO:0007803

R-HSA-1268020

R-HSA-2142789

R-HSA-196854

R-HSA-1430728

R-HSA-9609507

R-HSA-8978934

R-HSA-1299476

R-HSA-1299482

R-HSA-1299487

R-HSA-2162192

R-HSA-8986181

MONDO:0008922

R-HSA-1268020

R-HSA-83936

R-HSA-425407

R-HSA-425397

R-HSA-9609507

R-HSA-382551

R-HSA-1268022

R-HSA-1299481

R-HSA-1307803

R-HSA-1955380

R-HSA-5250209

R-HSA-5672027

MONDO:0009245

R-HSA-1268020

R-HSA-1362409

R-HSA-1430728

R-HSA-9609507

R-HSA-1299475

R-HSA-1299478

R-HSA-1299480

R-HSA-1362408

R-HSA-1362416

R-HSA-8986181

MONDO:0009505

R-HSA-1268020

R-HSA-9609507

R-HSA-1299476

R-HSA-1299482

R-HSA-1299487

R-HSA-8986181

MONDO:0010543

R-HSA-1268020

R-HSA-1482798

R-HSA-556833

R-HSA-1483206

R-HSA-1430728

R-HSA-9609507

R-HSA-1483257

R-HSA-1299481

R-HSA-1482781

R-HSA-1482794

R-HSA-1482850

R-HSA-1482894

R-HSA-1955380

MONDO:0010703

R-HSA-1268020

R-HSA-70635

R-HSA-71291

R-HSA-1430728

R-HSA-9609507

R-HSA-1299475

R-HSA-1299478

R-HSA-1299480

R-HSA-70560

R-HSA-8986181

MONDO:0011532

R-HSA-1268020

R-HSA-8869496

R-HSA-212436

R-HSA-74160

R-HSA-8864260

R-HSA-9609507

R-HSA-73857

R-HSA-1268022

R-HSA-1299475