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Water-soluble vitamins
Metabolism and transport of the B-vitamin and vitamin-C families. 146 reactions, from Reactome R-HSA-196849.
- MTR transfers CH3 from MeCbl to HCYSMTRR:MTR:MeCbl + HCYS → MTRR:MTR:cob(I)alamin + L-Met
- 2xPPCS ligates PPanK with CysPPanK + L-Cys + ATP → PPC + AMP + PPi + H+
- COASY transfers an adenylyl group from ATP to PPANTATP + H+ + PPANT → DP-CoA + PPi
- 2xTPK1:Mg2+ phosphorylates THMNTHMN + ATP → ThDP + AMP
- COASY phosphorylates DP-CoAATP + DP-CoA → ADP + CoA-SH + H+
- 3xPPCDC:3FMN decarboxylates PPCPPC + H+ → PPANT + CO2
- PANK2 phosphorylates PanKPanK + ATP → PPanK + ADP + H+
- FLAD1 phosphorylates FMNFMN + ATP + H+ → FAD + PPi
- 2xTRAP hydrolyzes FMN to RIBFMN + H2O → RIB + Pi
- 2xENPP1 hydrolyzes FAD to FMNFAD + H2O → AMP + FMN + 2 H+
- RFK:Mg2+ phosphorylates RIBRIB + ATP → FMN + ADP + H+
- NAPRT1 dimer transfers PRIB to NCA to form NAMNNCA + PRPP + ATP + H2O → PPi + NAMN + ADP + Pi
- ACS transforms to QUIN non-enzymaticallyACS → QUIN + H2O + H+
- NADK:Zn2+ tetramer phosphorylates NAD+ to NADP+NAD+ + ATP → NADP+ + ADP + H+
- NMNAT2 transfers an adenylyl group from ATP to NAMN to yield NAADATP + NAMN + H+ → NAAD + PPi
- NAMPT transfers PRIB to NAM to form NAMNNAM + PRPP + H+ → PPi + NAMN
- QPRT transfers PRIB to QUIN to form NAMNQUIN + PRPP + 2 H+ → PPi + CO2 + NAMN
- NADSYN1 hexamer amidates NAAD to NAD+NAAD + ATP + H2O + L-Gln → NAD+ + AMP + PPi + L-Glu + H+
- FPGS-2 transforms THF to THFPGTHF + 5 ATP + 5 L-Glu → THFPG + 5 ADP + 5 Pi
- DHFR2 reduces FOLA to DHFFOLA + NADPH + H+ → DHF + NADP+
- DHF is reduced to tetrahydrofolate (THF)DHF + NADPH + H+ → THF + NADP+
- GSTO dimers reduce DeHA to AscH-DeHA + 2 GSH → AscH- + GSSG
- SLC2A1,3 transports DeHA from extracellular region to cytosolDeHA → DeHA
- CYB5R3:FAD reduces CYB5A:ferriheme to CYB5A:hemeCYB5A:ferriheme + NADH + H+ → CYB5A:heme + NAD+
- CYB5A:heme reduces Asc.- to AscH-Asc.- + CYB5A:heme → AscH- + CYB5A:ferriheme
- SLC23A1,2 cotransports AscH-, 2Na+ from extracellular region to cytosolAscH- + 2 Na+ → AscH- + 2 Na+
- Phosphopantetheine conjugation of the ACP domain of FASCoA-SH + FASN → FASN + PAP
- PANK1/3 phosphorylate PanKPanK + ATP → PPanK + ADP + H+
- SLC5A6 cotransports extracellular PanK and 2Na+ to cytosolPanK + 2 Na+ → PanK + 2 Na+
- SLC25A16 transports cytosolic CoA-SH to mitochondrial matrixCoA-SH → CoA-SH
- SLC5A6 cotransports extracellular Btn and 2xNa+ to cytosolBtn + 2 Na+ → Btn + 2 Na+
- SLC19A2/3 transport extracellular THMN to cytosolTHMN → THMN
- NMNAT3 transfers an adenylyl group from ATP to NAMN to yield NAADNAMN + ATP + H+ → NAAD + PPi
- NMNAT1 transfers an adenylyl group from ATP to NAMN to yield NAADNAMN + ATP + H+ → NAAD + PPi
- 5,10-methyleneTHF polyglutamate + NADP+ 5,10-methenylTHF polyglutamate + NADPH + H+5,10-methylene-THFPG + NADP+ → 5,10-methenyl-THFPG + NADPH + H+
- Cytosolic folate export across the plasma membraneFOLA + H+ → FOLA + H+
- 5,10-methyleneTHF polyglutamate + glycine tetrahydrofolate polyglutamate (THF polyglutamate) + serine5,10-methylene-THFPG + Gly → THFPG + L-Ser
- SLC19A1 transports 5-methyl-THF from extracellular region to cytosol5-methyl-THF → 5-methyl-THF
- MTHFD1 dimer transforms 10-formyl-THFPG to 5,10-methenyl-THFPG10-formyl-THFPG → 5,10-methenyl-THFPG + H2O
- MTHFR dimer reduces 5,10-methylene-THFPG to 5-methyl-THFPG5,10-methylene-THFPG + NADPH + H+ → 5-methyl-THFPG + NADP+
- SLC25A32 transports THF from cytosol to mitochondrial matrixTHF → THF
- FPGS-2 transforms 5-methyl-THF to 5-methyl-THFPG5-methyl-THF + 5 ATP + 5 L-Glu → 5-methyl-THFPG + 5 ADP + 5 Pi
- Mitochondrial FPGS-1 transforms THF to THFPGTHF + 6 ATP + 6 L-Glu → THFPG + 6 ADP + 6 Pi
- MTHFD1 dimer ligates HCOOH to THFPG to form 10-formyl-THFPGTHFPG + HCOOH + ATP → 10-formyl-THFPG + ADP + Pi
- MTHFD1 dimer dehydrogenates 5,10-methenyl-THFPG to 5,10-methylene-THFPG5,10-methenyl-THFPG + NADPH + H+ → 5,10-methylene-THFPG + NADP+
- SLC25A32 transports THF from mitochondrial matrix to cytosolTHF → THF
- SLC46A1 transports FOLA from extracellular region to cytosolFOLA + H+ → FOLA + H+
- SHMT1 tetramer transfers hydroxymethyl group to THFPG to form 5,10-methylene-THFPGTHFPG + L-Ser → 5,10-methylene-THFPG + Gly
- 5,10-methenylTHF polyglutamate + H2O 10-formylTHF polyglutamate5,10-methenyl-THFPG + H2O → 10-formyl-THFPG
- HLCS biotinylates 6x(PCCA:PCCB)6x(PCCA:PCCB) + 6 Btn + 6 ATP → 6x(Btn-PCCA:PCCB) + 6 PPi + 6 AMP
- HLCS biotinylates 6xMCCC1:6xMCCC26xMCCC1:6xMCCC2 + 6 ATP + 6 Btn → 6x(Btn-MCCC1:MCCC2) + 6 PPi + 6 AMP
- HLCS biotinylates PC:Mn2+4x(PC:Mn2+) + 4 ATP + 4 Btn → 4x(Btn-PC:Mn2+) + 4 PPi + 4 AMP
- HLCS biotinylates ACACA:Mn2+ACACA:2Mn2+ + Btn + ATP → Btn-ACACA:2Mn2+ + AMP + PPi
- TCN2 binds RCbl in the circulationTCN2 + RCbl → TCN2:RCbl
- CUBN:AMN binds CBLIF:RCblCBLIF:RCbl + CUBN:AMN → CUBN:AMN:CBLIF:RCbl
- CD320-mediated TCN2:RCbl uptake and delivery to lysosomeTCN2:RCbl:CD320 → CD320 + TCN2:RCbl
- CBLIF binds RCblCBLIF + RCbl → CBLIF:RCbl
- CD320 binds extracellular TCN2:RCblCD320 + TCN2:RCbl → TCN2:RCbl:CD320
- CUBN:AMN-mediated CBLIF:RCbl uptake and delivery to lysosomeCUBN:AMN:CBLIF:RCbl → CBLIF:RCbl + CUBN:AMN
- Unknown lysosomal protease degrades CBLIF:RCbl to release CblCBLIF:RCbl → RCbl
- TCN2:RCbl is degraded to release RCblTCN2:RCbl → RCbl
- An unknown protease degrades ACACABtn-ACACA:2Mn2+ → BCTN
- An unknown protease degrades hCBXshCBXs → BCTN
- BCTN translocates to extracellular regionBCTN → BCTN
- Extracellular BTD hydrolyses BCTNBCTN → Btn + L-Lys
- MMACHC dealkylates RCblMMACHC + RCbl + GSH → R-GSH + MMACHC:cob(I)alamin + H+
- ABCC1 transports cytosolic RCbl to extracellular regionATP + H2O + RCbl → ADP + Pi + RCbl
- Pancreatic proteases degrade TCN1:RCblTCN1:RCbl → RCbl
- TCN1 binds RCblTCN1 + dietary protein:RCbl → TCN1:RCbl + dietary protein
- MMACHC:cob(II)alamin binds MMADHCMMACHC:cob(II)alamin + MMADHC → MMACHC:MMADHC:cob(II)alamin
- MTRR reduces cob(II)alamin to meCbl2 MTRR:MTR:cob(II)alamin + NADPH + 2 AdoMet → 2 MTRR:MTR:MeCbl + 2 AdoHcy + NADP+ + H+
- MMACHC decyanates CNCbl2 CNCbl + NADPH + H+ + 2 MMACHC → 2 MMACHC:cob(II)alamin + 2 HCN + NADP+
- MTR transfers CH3 group from 5-methyl-THF to cob(I)alaminMTRR:MTR:cob(I)alamin + 5-methyl-THF → MTRR:MTR:MeCbl + THF
- MMADHC targets transport of cytosolic cob(II)alamin to mitochondriaMMACHC:MMADHC:cob(II)alamin → cob(II)alamin + MMADHC + MMACHC
- MMAB adenosylates cob(I)alamin2 ATP + 2 cob(II)alamin + FADH2 → 2 PPP + 2 AdoCbl + 3 H+ + FAD
- MMAA:MUT binds AdoCbl2xMMAA:2xMUT + AdoCbl → 2xMMAA:2xMUT:AdoCbl
- SLC52A1,2,3 transport RIB from extracellular region to cytosolRIB → RIB
- AOX1 oxidises PXL to PDXateH2O + O2 + PXL → PDXate + H2O2
- cob(II)alamin is transferred from MMACHC:MMADHC:cob(II)alamin to MTRR:MTRMTRR:MTR + MMACHC:MMADHC:cob(II)alamin → MTRR:MTR:cob(II)alamin + MMACHC + MMADHC
- TCN1 binds correnoids in the circulationTCN1 + correnoid → TCN1:correnoid
- Cytosolic carboxylases translocate to mitochondrial matrixhCBXs → hCBXs
- An unknown protease degrades ACACBBtn-ACACB:2Mn2+ → BCTN
- Mitochondrial BTD hydrolyses BCTNBCTN → Btn + L-Lys
- HLCS biotinylates ACACBACACB:2Mn2+ + Btn + ATP → Btn-ACACB:2Mn2+ + AMP + PPi
- SLC5A8 (SMCT) co-transports monocarboxylates and Na+ from extracellular region to cytosolmonocarboxylates transported by SLC5A8 + 2 Na+ → monocarboxylates transported by SLC5A8 + 2 Na+
- ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells)ATP + H2O + RCbl → ADP + Pi + RCbl
- NNMT transfers CH3 from SAM to NAM to form MNANAM + AdoMet → AdoHcy + MNA
- PXLP-K280-SHMT2 tetramer transfers CH2OH group to Gly to form L-Ser5,10-methylene-THF + Gly + H2O → THF + L-Ser
- MTHFD1L dimer ligates HCOOH to THF to form 10-formyl-THFTHF + HCOOH + ATP → 10-formyl-THF + ADP + Pi
- MTHFD2, D2L oxidise 5,10-methylene-THF to 5,10-methenyl-THF5,10-methylene-THF + NAD+ → 5,10-methenyl-THF + NADH
- MTHFS transforms 5-formyl-THFPG to 5,10-methenyl-THFPG5-formyl-THFPG + ATP → 5,10-methenyl-THFPG + ADP + Pi + H+
- MTHFD1L transforms 10-formyl-THF to HCOOH and THF10-formyl-THF + Pi + ADP → HCOOH + THF + ATP
- MTHFD2, D2L oxidise 5,10-methenyl-THF to 10-formyl-THF5,10-methenyl-THF + H2O → 10-formyl-THF
- HCOOH translocated from mitochondrial matrix to cytosolHCOOH → HCOOH
- APOA1BP dimer epimerises R-NAD(P)HX to S-NAD(P)HXR-NADPHX → S-NADPHX
- CARKD dehydrates S-NAD(P)HX to NADPHS-NADPHX + ATP → NADPH + ADP + Pi + 3 H+
- ALDH1L2 dehydrogenates 10-formyl-THFPG to THFPG10-formyl-THFPG + NADP+ + H2O → THFPG + NADPH + H+ + CO2
- ALDH1L1 dehydrogenates 10-formyl-THFPG to THFPG10-formyl-THFPG + NADP+ + H2O → THFPG + NADPH + H+ + CO2
- NUDT12 hydrolyses NADH to NMNHNADH + H2O → NMNH + 2 H+ + AMP
- SLC22A13 exchanges extracellular NCA for cytosolic urateNCA + urate → NCA + urate
- NMRK1 phosphorylates NAR to yield NAMNNAR + ATP → ADP + H+ + NAMN
- NMRK2 phosphorylates NAR to yield NAMNNAR + ATP → ADP + H+ + NAMN
- NMRK2 phosphorylates NR to yield NMNNR + ATP → NMN + ADP + H+
- NMRK1 phosphorylates NR to yield NMNNR + ATP → NMN + ADP + H+
- BST1 hydrolyzes NAD+ to yield NAM and ADP-riboseNAD+ + H2O → NAM + ADP-ribose + H+
- SLC25A19 transports ThDP from cytosol to mitochondrial matrixThDP → ThDP
- PARPs transfer ADP-D-ribose to proteins (poly(ADP-ribosyl)ation)3 NAD+ + acceptor protein → 3 NAM + ADP-D-ribosyl-acceptor protein
- CD38 hydrolyses NAD+ to NAM and ADP-riboseNAD+ + H2O → NAM + ADP-ribose + H+
- Vanin hydrolyses pantetheine to PanK, 2AETPantetheine → 2AET + PanK
- ENPPs hydrolyse CoA-SH to PPANT, PAPCoA-SH + H2O → PPANT + PAP
- PPANT translocates from extracellular region to cytosolPPANT → PPANT
- NMNAT2 transfers an adenylyl group from ATP to NMN to yield NAD+NMN + ATP + H+ → NAD+ + PPi
- NT5E:Zn2+ hydrolyses NAD+H2O + NAD+ → NMN + Pi + AMP + 2 H+
- NT5E:Zn2+ hydrolyses NMNH2O + NMN → NR + Pi
- FOLR2 binds FOLAFOLA + FOLR2 → FOLR2:FOLA
- FOLR2 transports FOLA from extracellular region to cytosolFOLR2:FOLA → FOLR2 + FOLA
- NADK2 dimer phosphorylates NAD+ to NADP+NAD+ + ATP → NADP+ + ADP + H+
- RNLS:FAD oxidises dh-beta-NAD to NAD+dh-beta-NAD + O2 + H+ → NAD+ + H2O2
- Exchange of oxygen with sulfur in MoCoL-Cys + MoCo → L-Ala + sulfurated MoCo
- PXLP-K198-NFS1 transfers sulfur from cysteine onto MOCS3L-Cys + MOCS3:Zn2+ (red.) → L-Ala + MOCS3-S-S(1-):Zn2+
- Molybdenum ion transfer onto molybdopterinMPT + MoO4(2-) + ATP + H2O → MoCo + AMP + PPi
- Cyclisation of GTP to precursor ZGTP + AdoMet + H2O → Precursor Z + PPi + L-Met + dADE
- Transfer of sulfur from MOCS3-S-S onto MOCS2AMOCS2 + ATP + MOCS3-S-S(1-):Zn2+ → SOG-MOCS2 + AMP + PPi + MOCS3:Zn2+ (ox.)
- Sulfhydrylation and ring cleavage of precursor ZPrecursor Z + H2O + 2xMOCS2-CO-S(1-):2xMOCS2B → MPT + H+ + 2xMOCS2A:2xMOCS2B
- Asc.- radical dissociates to AscH- and DeHA2 Asc.- + H+ → AscH- + DeHA
- DeHA hydrolyses to 2,3-DKGDeHA + H2O → 2,3-DKG
- DeHA hydrolyses to threonate and oxalateDeHA + H2O + H2O2 → oxalate + threonate
- 2,3-DKG hydrolyses to ERU and oxalate2,3-DKG + H2O → ERU + oxalate + H+
- 2xPDXK:2xZn2+ phosphorylates PXAPXA + ATP → PXAP + ADP
- 2xPDKX:2xZn2+ phosphorylates PDXPDX + ATP → PDXP + ADP
- 2xPDXK:2xZn2+ phosphorylates PXLPXL + ATP → PXLP + ADP
- 2xPNPO:2xFMN oxidizes PDXP to PXLPPDXP + O2 → PXLP + H2O2
- THTPA:Mg2+ hydrolyzes ThTP to TDPThTP + H2O → ThDP + Pi
- 2xPNPO:2xFMN oxidizes PXAP to PXLPPXAP + H2O + O2 → PXLP + NH4+ + H2O2
- DHFR dimer reduces FOLA to DHFFOLA + NADPH + H+ → DHF + NADP+
- DHFR dimer binds DHFR inhibitorsDHFR dimer + DHFR inhibitors → DHFR dimer:DHFR inhibitors
- LRP2-mediated TCN2:RCbl uptake and delivery to lysosomeLRP2:LDLRAP1:TCN2:RCbl → TCN2:RCbl + LRP2:LDLRAP1
- ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosolATP + H2O + RCbl → ADP + Pi + RCbl
- LRP2 binds extracellular TCN2:RCblTCN2:RCbl + LRP2:LDLRAP1 → LRP2:LDLRAP1:TCN2:RCbl
- Cob(I)alamin bound to MMACHC is oxidized to cob(II)alamin2 MMACHC:cob(I)alamin + H+ + NAD+ → 2 MMACHC:cob(II)alamin + NADH
- SLC25A42 exchanges cytosolic CoA-SH for mitochondrial matrix ADPADP + CoA-SH → ADP + CoA-SH
- DCAKD phosphorylates DP-CoAATP + DP-CoA → ADP + CoA-SH + H+
- NUDT8 hydrolyzes CoA-SH to PPANTCoA-SH + H2O → 2 H+ + PAP + PPANT
- PANK4 hydrolyzes PPANT to pantetheinePPANT + H2O → Pantetheine + Pi
- SLC25A51 mediates mitochondrial uptake of NAD+NAD+ → NAD+
- TDPK phosphorylates ThDP to ThTPATP + ThDP → ADP + ThTP