GDA
SLC25A16
UniProt:P16260 SLC25A16
ABCC1
MRP
MRP1
UniProt:P33527 ABCC1
FLOT1
RFC1
SLC19A1
UniProt:P41440 SLC19A1
BTD
UniProt:P43251 BTD
NAMPT
PBEF
PBEF1
UniProt:P43490 NAMPT
HLCS
UniProt:P50747 HLCS
FPGS
UniProt:Q05932-1 FPGS
UniProt:Q05932-2 FPGS
COASY
PSEC0106
UniProt:Q13057 COASY
QPRT
UniProt:Q15274 QPRT
FLAD1
PP591
UniProt:Q8NFF5 FLAD1
HCP1
PCFT
SLC46A1
UniProt:Q96NT5 SLC46A1
C20orf48
PANK2
UniProt:Q9BZ23 PANK2
MFT
MFTC
SLC25A32
UniProt:Q9H2D1 SLC25A32
8D6A
CD320
UniProt:Q9NPF0 CD320
C20orf54
RFT2
SLC52A3
UniProt:Q9NQ40 SLC52A3
AASDHPPT
CGI-80
HAH-P
HSPC223
UniProt:Q9NRN7 AASDHPPT
BM-021
C6orf209
CD001
LMBRD1
MSTP044
NESI
UniProt:Q9NUN5 LMBRD1
SLC5A6
SMVT
UniProt:Q9Y289 SLC5A6
MMACHC
UniProt:Q9Y4U1 MMACHC
L-methylmalonyl-CoA <=> succinyl-CoA
MUT transfers adenosyl group from AdoCbl to L-MM-CoA
Dehydroascorbate transport across the plasma membrane
GLUT1/3 transports extracellular DHvitC to cytosol
dehydroascorbate [extracellular] => dehydroascorbate [cytosol]
Ascorbate transport across the plasma membrane
SVCT1/2 cotransports extracellular VitC and 2Na+ to cytosol
ascorbate [extracellular] + 2 Na+ [extracellular] => ascorbate [cytosol] + 2 Na+ [cytosol]
CYB5A:heme reduces SDA to VitC
Reduction of semidehydroascorbate to ascorbate
CYB5R3:FAD reduces CYB5A:ferriheme to CYB5A:heme
ferric CYB5A + NADH + H+ => ferrous CYPB5A + NAD+
2xGSTOs reduce DHvitC to VitC
dehydroascorbate (DHA) + 2 glutathione (GSH) => ascorbate + oxidized glutathione (GSSG)
SLC19A2/3 transport extracellular THMN to cytosol
Thiamin transport across the plasma membrane
thiamin [extracellular] => thiamin [cytosol]
2xTPK1:Mg2+ phosphorylates THMN
Thiamin is pyrophosphorylated
TDPK phosphorylates ThDP to ThTP
ThDP + ATP <-> ThTP + ADP
THTPA:Mg2+ hydrolyzes ThTP to TDP
Thiamin triphosphate can be hydrolysed back to thiamin diphosphate
SLC52A3 transports extracellular RIB to cytosol
RFK:Mg2+ phosphorylates RIB
FLAD1 phosphorylates FMN
FMN is futher phosphorylated to FAD
2xENPP1 hydrolyzes FAD to FMN
FAD can be hydrolyzed back to FMN
2xTRAP hydrolyzes FMN to RIB
FMN can be hydrolyzed back to riboflavin
SLC5A6 cotransports extracellular PanK and 2Na+ to cytosol
pantothenate [extracellular] + 2 Na+ [extracellular] => pantothenate [cytosol] + 2 Na+ [cytosol]
PANK2 phosphorylates PanK
Pantothenate is phosphorylated [PANK2]
PANK1/3/4 phosphorylate PanK
Pantothenate is phosphorylated [PANK1;3;4]
pantothenate + ATP => phosphopanthothenate + ADP
2xPPCS ligates PPanK with Cys
Condensation of phosphopantothenate with cysteine
3xPPCDC:3FMN decarboxylates PPC
Phosphopantothenoylcysteine is decarboxylated
Adenylation of phosphopantetheine
COASY transfers an adenylyl group from ATP to PPANT
COASY phosphorylates DP-CoA
Phosphorylation of dephospho-CoA to produce CoA
CoA transport across the inner mitochondrial membrane
SLC25A16 transports cytosolic CoA-SH to mitichondrial matrix
CoA-SH + FAS apoenzyme => adenosine 3',5'-bisphosphate + FAS holoenzyme
Phosphopantetheine conjugation of the ACP domain of FAS
2xPDXK:2xZn2+ phosphorylates PXL
PLP results from phosphorylation of pyridoxal
2xPDXK:2xZn2+ phosphorylates PXA
Pyridoxamine is phosphorylated
2xPDKX:2xZn2+ phosphorylates PDX
Pyridoxine is phosphorylated
2xPNPO:2xFMN oxidizes PXAP to PXLP
PLP from oxidation of pyridoxamine phosphate
2xPNPO:2xFMN oxidizes PDXP to PXLP
PLP from oxidation of pyridoxine phosphate
AOX1 oxidises PXL to PDXate
TCN1 binds Cbl
Pancreatic proteases degrade TCN1:Cbl
GIF binds Cbl
CUBN:AMN binds GIF:Cbl
CUBN:AMN transports extracellular GIF:Cbl to endosome
Endosomal GIF:Cbl translocates to lysosome
GIF:Cbl is degraded to release Cbl
LMBRD1 transports lysosomal Cbl to cytosol
MMACHC reduces Cbl
MMACHC decyanates CNCbl
MMACHC reductively decyanates CNCbl
MMACHC:B12r binds MMADHC
B12r dissociates from MMACHC:MMADHC
B12r binds to MTRR:MTR
MTRR reduces B12r to B12s
MTR transfers CH3 group from MTHF to B12s
MTR transfers CH3 group from MeCbl to HCYS
Methionine is reformed
MMADHC targets cytosolic B12r for entry to mitochondria
MMADHC targets transport of cytosolic B12r to mitochondria
MMAB reduces B12r to B12s
MMAB transfers adenosyl group from ATP to B12s
MMAA:MUT binds AdoCbl
ABCC1 transports cytosolic Cbl to extracellular region
TCN2 binds Cbl in the circulation
TCN1 binds Cbl in the circulation
CD320 transports extracellular TCN2:Cbl to endosome
CD320:TCN2:Cbl dissociates into CD320 and TCN2:Cbl
Endosomal TCN2:Cbl translocates to lysosome
TCN2:Cbl is degraded to release Cbl
SLC5A6 cotransports extracellular Btn and 2xNa+ to cytosol
biotin [extracellular] + 2 Na+ [extracellular] => biotin [cytosol] + 2 Na+ [cytosol]
HLCS biotinylates PCCA:PCCB
HLCS biotinylates PC:Mn2+
HLCS biotinylates MCCC1:MCCC2
Cytoslic carboxylases translocate to mitochondrial matrix
HLCS biotinylates ACACA/B:Mn2+
An unknown protease degrades hCBXs
An unknown protease degrades ACACA/B:Mn2+
BCTN translocates to extracellular region
BTD hydrolyses BCTN
2-amino 3-carboxymuconate semialdehyde transforms non-enzymatically to quinolinate
A phospho-ribosyl group is added to quinolinate
Nicotinate D-ribonucleotide + ATP => deamino-NAD+ + pyrophosphate [NMNAT1]
Nicotinate D-ribonucleotide + ATP => deamino-NAD+ + pyrophosphate [NMNAT2]
Nicotinate D-ribonucleotide + ATP => deamino-NAD+ + pyrophosphate [NMNAT3]
Amidation of deamino-NAD+ to NAD+
NAD+ is phosphorylated to NADP+
Deamination of nicotinamide to nicotinate
Condensation of nicotinamide to nicotinamide D-ribonucleotide (NMN)
A phosphoribosyl group is added to nicotinate to form nicotinate mononucleotide (NaMN)
Extracellular folate import across the plasma membrane
Cytosolic folate export across the plasma membrane
Extracellular 5-methyltetrahydrofolate import across the plasma membrane
Folate is reduced to dihydrofolate (DHF)
DHF is reduced to tetrahydrofolate (THF)
Conversion of cytosolic THF to THF-polyglutamate
Conversion of cytosolic 5-methyltetrahydrofolate (5-methylTHF) to 5-methylTHF-polyglutamate
Cytosolic tetrahydrofolate import across the inner mitochondrial membrane
Mitochondrial tetrahydrofolate export across the inner mitochondrial membrane
Conversion of mitochondrial THF to THF-polyglutamate
THF polyglutamate + formate + ATP => 10-formylTHF polyglutamate + ADP + orthophosphate
10-formylTHF polyglutamate <=> 5,10-methenylTHF polyglutamate + H2O
5,10-methenylTHF polyglutamate + NADPH + H+ <=> 5,10-methyleneTHF polyglutamate + NADP+
Tetrahydrofolate polyglutamate (THF polyglutamate) + serine <=> 5,10-methyleneTHF polyglutamate + glycine
5,10-methyleneTHF polyglutamate + NADPH + H+ => 5-methylTHF polyglutamate + NADP+
5,10-methyleneTHF polyglutamate + glycine <=> tetrahydrofolate polyglutamate (THF polyglutamate) + serine
5,10-methyleneTHF polyglutamate + NADP+ <=> 5,10-methenylTHF polyglutamate + NADPH + H+
5,10-methenylTHF polyglutamate + H2O <=> 10-formylTHF polyglutamate
Cyclisation of GTP to precursor Z
NFS1 transfers sulfur from cysteine onto MOCS3
Transfer of sulfur from MOCS3-S-S onto MOCS2A
Sulfhydrylation and ring cleavage of precursor Z
Molybdenum ion transfer onto molybdopterin
Exchange of oxygen with sulfur in MoCo
Metabolism of vitamins and cofactors
Metabolism of water-soluble vitamins and cofactors
Vitamin C (ascorbate) metabolism
Vitamin B1 (thiamin) metabolism
Vitamin B2 (riboflavin) metabolism
Vitamin B5 (pantothenate) metabolism
Coenzyme A biosynthesis
Vitamins B6 activation to pyridoxal phosphate
Cobalamin (Cbl, vitamin B12) transport and metabolism
Biotin transport and metabolism
Nicotinate metabolism
Nicotinamide salvaging
Metabolism of folate and pterines
Molybdenum cofactor biosynthesis