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Proton-coupled folate transporter Dihydrofolate reductase Dihydrofolate reductase C-1- tetrahydrofolate synthase, cytoplasmic Cytosolic 10- formyltetrahydrofolate dehydrogenase Formimidoyltransferase- cyclodeaminase C-1- tetrahydrofolate synthase, cytoplasmic 5- formyltetrahydrofolate cyclo-ligase C-1- tetrahydrofolate synthase, cytoplasmic Methylenetetrahydrofolate reductase Formimidoyltransferase- cyclodeaminase Folylpolyglutamate synthase, mitochondrial Folylpolyglutamate synthase, mitochondrial Gamma-glutamyl hydrolase Monofunctional C1- tetrahydrofolate synthase, mitochondrial Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase, mitochondrial Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase, mitochondrial Methionyl-tRNA formyltransferase, mitochondrial Tetrahydrofolic acid Dihydrofolic acid L-Glutamic acid 5-Formiminotetrahydrofolic acid 5,10-Methylene-THF 5-Methyltetrahydrofolic acid 10-Formyltetrahydrofolate Tetrahydrofolic acid 5,10-Methylene-THF Folic acid Folic acid NADP NADPH NADP NADPH ATP Formic acid ADP Pi NADP H2O CO2 NADPH N5-Formyl-THF N-Formyl-L-glutamic acid H+ 5,10-Methenyltetrahydrofolic acid H2O ATP ADP Pi NADPH NADP NADH NAD NH3 L-Glutamic acid ATP Tetrahydrofolyl-[Glu](2) ADP Pi ATP L-Glutamic acid Tetrahydrofolyl-[Glu](n) ADP Pi H2O L-Glutamic acid ATP Formic acid ADP 10-Formyltetrahydrofolate Pi H+ 5,10-Methenyltetrahydrofolic acid H2O NADH NAD Pyridoxal 5'-phosphate Magnesium Pyridoxal 5'-phosphate Calcium Magnesium Magnesium L- Methionyl- tRNA(Met) N- formylmethionyl- tRNA(fMet) Histidine Metabolism Purine Metabolism Methionine Metabolism Glycine and Serine Metabolism Pyrimidine Metabolism Glutamate Metabolism Histidine Metabolism Glycine and Serine Metabolism Pyrimidine Metabolism Methionine Metabolism Purine Metabolism Glycine and Serine Metabolism Glycine and Serine Metabolism FAD Calcium Mitochondria Lysosome Extracellular Space Intracellular Space Mitochondrial matrix Red Blood Cell Symptoms include feeding difficulties and failure to thrive and anemia, as well as potential neurological issues such as seizures and developmental delays. Brain
Mitochondria SLC46A1 DHFR DHFR MTHFD1 ALDH1L1 FTCD MTHFD1 MTHFS MTHFD1 MTHFR FTCD FPGS FPGS GGH MTHFD1L MTHFD2 MTHFD2 MTFMT Tetrahydrofolic acid Dihydrofolic acid L-Glutamic acid 5- Formiminotetrahydrofolic acid 5,10- Methylene-THF 5- Methyltetrahydrofolic acid 10- Formyltetrahydrofolate Tetrahydrofolic acid 5,10- Methylene-THF Folic acid Folic acid NADP NADPH NADP NADPH Adenosine triphosphate Formic acid Adenosine diphosphate Phosphate NADP Water Carbon dioxide NADPH N5-Formyl-THF N-Formyl-L- glutamic acid Hydrogen Ion 5,10- Methenyltetrahydrofolic acid Water Adenosine triphosphate Adenosine diphosphate Phosphate NADPH NADP NADH NAD Ammonia L-Glutamic acid Adenosine triphosphate Tetrahydrofolyl- [Glu](2) Adenosine diphosphate Phosphate Adenosine triphosphate L-Glutamic acid Tetrahydrofolyl- [Glu](n) Adenosine diphosphate Phosphate Water L-Glutamic acid Adenosine triphosphate Formic acid Adenosine diphosphate 10- Formyltetrahydrofolate Phosphate Hydrogen Ion 5,10- Methenyltetrahydrofolic acid Water NADH NAD L- Methionyl- tRNA(Met) N- formylmethionyl- tRNA(fMet) Histidine Metabolism Purine Metabolism Methionine Metabolism Glycine and Serine Metabolism Pyrimidine Metabolism Glutamate Metabolism Histidine Metabolism Glycine and Serine Metabolism Pyrimidine Metabolism Methionine Metabolism Purine Metabolism Glycine and Serine Metabolism Glycine and Serine Metabolism
SLC46A1 DHFR DHFR MTHFD1 ALDH1L1 FTCD MTHFD1 MTHFS MTHFD1 MTHFR FTCD FPGS FPGS GGH MTHFD1L MTHFD2 MTHFD2 MTFMT THFA FAH2 Glu 5-FTHA 5XM-THF 5-MTHFa 10-FTHF THFA 5XM-THF Folate Folate NADP NADPH NADP NADPH ATP Formate ADP Pi NADP H2O CO2 NADPH Leucal NFLGA H+ CH-THF H2O ATP ADP Pi NADPH NADP NADH NAD NH3 Glu ATP THFYL ADP Pi ATP Glu ThGluN ADP Pi H2O Glu ATP Formate ADP 10-FTHF Pi H+ CH-THF H2O NADH NAD Pyr-5'P Mg2+ Pyr-5'P Ca2+ Mg2+ Mg2+ LMTM NFTF PW000043 PW000052 PW000056 PW000157 PW000160 PW000003 PW000043 PW000157 PW000160 PW000056 PW000052 PW000157 PW000157 FAD Ca2+ Mitochondria Lysosome Extracellular Space Intracellular Space Mitochondrial matrix Red Blood Cell Symptoms include feeding difficulties and failure to thrive and anemia, as well as potential neurological issues such as seizures and developmental delays. Brain
Mitochondria SLC46A1 DHFR DHFR MTHFD1 ALDH1L1 FTCD MTHFD1 MTHFS MTHFD1 MTHFR FTCD FPGS FPGS GGH MTHFD1L MTHFD2 MTHFD2 MTFMT THFA FAH2 Glu 5-FTHA 5XM-THF 5-MTHFa 10-FTHF THFA 5XM-THF Folate Folate NADP NADPH NADP NADPH ATP Formate ADP Pi NADP H2O CO2 NADPH Leucal NFLGA H+ CH-THF H2O ATP ADP Pi NADPH NADP NADH NAD NH3 Glu ATP THFYL ADP Pi ATP Glu ThGluN ADP Pi H2O Glu ATP Formate ADP 10-FTHF Pi H+ CH-THF H2O NADH NAD LMTM NFTF PW000043 PW000052 PW000056 PW000157 PW000160 PW000003 PW000043 PW000157 PW000160 PW000056 PW000052 PW000157 PW000157