Label Type Identifier Comment Ensembl NCBI gene HGNC UniProt Wikidata ChEBI InChI (S)-Beta-aminoisobutyrate Metabolite chebi:57731 "aka (R)-3-amino-2-methylpropanoate; myokine, β-aminoisobutyric acid (BAIBA); D form of β-aminoisobutyric acid (D-BAIBA)
Zwitterion needed for conversion to take place" wikidata:Q106345514 chebi:57731;chebi:57731 inchikey:QCHPKSFMDHPSNR-GSVOUGTGSA-N Glutamine Metabolite chebi:58359 "Zwitterion needed for conversion to take place" wikidata:Q106345471 chebi:58359;chebi:58359 inchikey:ZDXPYRJPNDTMRX-VKHMYHEASA-N Orotate Metabolite chebi:30839 "" wikidata:Q27104157 chebi:30839;chebi:30839 inchikey:PXQPEWDEAKTCGB-UHFFFAOYSA-M UTP Metabolite chebi:46398 "(4-) charge needed for conversion to take place" wikidata:Q27104565 chebi:46398;chebi:46398 inchikey:PGAVKCOVUIYSFO-XVFCMESISA-J Carbamoyl-phosphate Metabolite chebi:58228 "(2-) charge needed for conversion to take place" wikidata:Q27125628 chebi:58228;chebi:58228 inchikey:FFQKYPRQEYGKAF-UHFFFAOYSA-L L-Valine Metabolite chebi:57762 "" wikidata:Q106345667 chebi:57762;chebi:57762 inchikey:KZSNJWFQEVHDMF-BYPYZUCNSA-N CoQ10 Metabolite chebi:46245 "" wikidata:Q321285 chebi:46245;chebi:46245 inchikey:ACTIUHUUMQJHFO-UPTCCGCDSA-N Thymidine Metabolite chebi:17748 "" wikidata:Q422464 chebi:17748;chebi:17748 inchikey:IQFYYKKMVGJFEH-XLPZGREQSA-N OMP Metabolite chebi:57538 "Orotidylic acid
(3-) charge needed for conversion to take place" wikidata:Q27124764 chebi:57538;chebi:57538 inchikey:KYOBSHFOBAOFBF-XVFCMESISA-K Acetyl-CoA Metabolite chebi:15351 "" wikidata:Q715317 chebi:15351;chebi:15351 inchikey:ZSLZBFCDCINBPY-ZSJPKINUSA-N Aspartate Metabolite chebi:29991 "(1-) charge needed for conversion to take place" wikidata:Q27104098 chebi:29991;chebi:29991 inchikey:CKLJMWTZIZZHCS-REOHCLBHSA-M Carbamoylaspartate Metabolite chebi:32814 "aka N-carbamoyl-L-aspartate
(2-) charge needed for conversion to take place" wikidata:Q27104235 chebi:32814;chebi:32814 inchikey:HLKXYZVTANABHZ-REOHCLBHSA-L CDP Metabolite chebi:58069 "(3-) charge needed for conversion to take place" wikidata:Q27125163 chebi:58069;chebi:58069 inchikey:ZWIADYZPOWUWEW-XVFCMESISA-K N-Carbamoyl-beta-alanine Metabolite chebi:11892 "aka 3-(carbamoylamino)propanoate" wikidata:Q27108902 chebi:11892;chebi:11892 inchikey:JSJWCHRYRHKBBW-UHFFFAOYSA-M Uridine Metabolite chebi:16704 "" wikidata:Q422573 chebi:16704;chebi:16704 inchikey:DRTQHJPVMGBUCF-XVFCMESISA-N CoQ10 - H2 (reduced) Metabolite chebi:64183 "" wikidata:Q28529714 chebi:64183;chebi:64183 inchikey:QNTNKSLOFHEFPK-UPTCCGCDSA-N Ornithine Metabolite chebi:46911 "(1) charge needed for conversion to take place" wikidata:Q27120725 chebi:46911;chebi:46911 inchikey:AHLPHDHHMVZTML-BYPYZUCNSA-O Dihydrouracil Metabolite chebi:15901 "" wikidata:Q409340 chebi:15901;chebi:15901 inchikey:OIVLITBTBDPEFK-UHFFFAOYSA-N Cytidine Metabolite chebi:17562 "" wikidata:Q422538 chebi:17562;chebi:17562 inchikey:UHDGCWIWMRVCDJ-XVFCMESISA-N Dihydrothymine Metabolite chebi:27468 "" wikidata:Q3027885 chebi:27468;chebi:27468 inchikey:NBAKTGXDIBVZOO-UHFFFAOYSA-N UMP Metabolite chebi:57865 "(2-) charge needed for conversion to take place" wikidata:Q27125012 chebi:57865;chebi:57865 inchikey:DJJCXFVJDGTHFX-XVFCMESISA-L Citrulline Metabolite chebi:57743 "Zwitterion needed for conversion to take place" wikidata:Q106345633 chebi:57743;chebi:57743 inchikey:RHGKLRLOHDJJDR-BYPYZUCNSA-N dUDP Metabolite chebi:60471 "(3-) charge needed for conversion to take place" wikidata:Q27127304 chebi:60471;chebi:60471 inchikey:QHWZTVCCBMIIKE-SHYZEUOFSA-K Malonate semialdehyde Metabolite chebi:57700 "AKA Beta-ketopropionate
Annotated without knowledge of R/S configuration" wikidata:Q27072236 chebi:57700;chebi:57700 inchikey:VOKUMXABRRXHAR-UHFFFAOYSA-M dUMP Metabolite chebi:246422 "(2-) charge needed for conversion to take place" wikidata:Q27225714 chebi:246422;chebi:246422 inchikey:JSRLJPSBLDHEIO-SHYZEUOFSA-L D-methylmalonatesemialdehyde Metabolite chebi:141212 "methylmalonate semialdehyde aka 2-Methyl-3-oxopropanoic acid aka 2-methyl-3-oxopropanoate
D configuration == R-annotation in ChEBI" wikidata:Q74411260 chebi:141212;chebi:141212 inchikey:VOKUMXABRRXHAR-GSVOUGTGSA-M Uracil Metabolite chebi:17568 "" wikidata:Q182990 chebi:17568;chebi:17568 inchikey:ISAKRJDGNUQOIC-UHFFFAOYSA-N CMP Metabolite chebi:60377 "(2-) charge needed for conversion to take place" wikidata:Q27104867 chebi:60377;chebi:60377 inchikey:IERHLVCPSMICTF-XVFCMESISA-L Orotidine Metabolite chebi:25722 "" wikidata:Q10859719 chebi:25722;chebi:25722 inchikey:FKCRAVPPBFWEJD-XVFCMESISA-N N-Carbamyl-beta-aminoisobutyric acid Metabolite chebi:74414 "aka 3-(carbamoylamino)-2-methylpropanoate
(1-) charge needed for conversion to take place" wikidata:Q27144658 chebi:74414;chebi:74414 inchikey:PHENTZNALBMCQD-UHFFFAOYSA-M CTP Metabolite chebi:37563 "(4-) charge needed for conversion to take place" wikidata:Q27117190 chebi:37563;chebi:37563 inchikey:PCDQPRRSZKQHHS-XVFCMESISA-J Beta-alanine Metabolite chebi:57966 "Zwitterion needed for conversion to take place" wikidata:Q106345620 chebi:57966;chebi:57966 inchikey:UCMIRNVEIXFBKS-UHFFFAOYSA-N PRPP Metabolite chebi:17111 "" wikidata:Q420633 chebi:17111;chebi:17111 inchikey:PQGCEDQWHSBAJP-TXICZTDVSA-N UDP Metabolite chebi:58223 "(3-) charge needed for conversion to take place" wikidata:Q27125623 chebi:58223;chebi:58223 inchikey:XCCTYIAWTASOJW-XVFCMESISA-K Uridine Metabolite chebi:16704 "" wikidata:Q422573 chebi:16704;chebi:16704 inchikey:DRTQHJPVMGBUCF-XVFCMESISA-N Thymine Metabolite chebi:17821 "" wikidata:Q171973 chebi:17821;chebi:17821 inchikey:GCIVSXAHMONYLO-HMAFJQTKSA-N;inchikey:RWQNBRDOKXIBIV-UHFFFAOYSA-N HCO3- Metabolite chebi:17544 "" wikidata:Q409202 chebi:17544;chebi:17544 inchikey:BVKZGUZCCUSVTD-UHFFFAOYSA-M 2-Deoxyuridine Metabolite chebi:16450 "" wikidata:Q419140 chebi:16450;chebi:16450 inchikey:MXHRCPNRJAMMIM-SHYZEUOFSA-N dTMP Metabolite chebi:63528 "(2-) charge needed for conversion to take place" wikidata:Q743705 chebi:63528;chebi:63528 inchikey:GYOZYWVXFNDGLU-XLPZGREQSA-L Orotate Metabolite chebi:30839 "" wikidata:Q27104157 chebi:30839;chebi:30839 inchikey:PXQPEWDEAKTCGB-UHFFFAOYSA-M Dihydroorotate Metabolite chebi:30864 "" wikidata:Q27104161 chebi:30864;chebi:30864 inchikey:UFIVEPVSAGBUSI-REOHCLBHSA-M Carbamoyl-phosphate Metabolite chebi:58228 "(2-) charge needed for conversion to take place" wikidata:Q27125628 chebi:58228;chebi:58228 inchikey:FFQKYPRQEYGKAF-UHFFFAOYSA-L Glutamine Metabolite chebi:58359 "Zwitterion needed for conversion to take place" wikidata:Q106345471 chebi:58359;chebi:58359 inchikey:ZDXPYRJPNDTMRX-VKHMYHEASA-N HCO3- Metabolite chebi:17544 "" wikidata:Q409202 chebi:17544;chebi:17544 inchikey:BVKZGUZCCUSVTD-UHFFFAOYSA-M + PRPP Metabolite chebi:17111 "" wikidata:Q420633 chebi:17111;chebi:17111 inchikey:PQGCEDQWHSBAJP-TXICZTDVSA-N Glutamate Metabolite chebi:29985 "(1-) charge needed for conversion to take place" wikidata:Q27104095 chebi:29985;chebi:29985 inchikey:WHUUTDBJXJRKMK-VKHMYHEASA-M NH4+ Metabolite chebi:28938 "" wikidata:Q190901 chebi:28938;chebi:28938 inchikey:QGZKDVFQNNGYKY-UHFFFAOYSA-O Dihydroorotate Metabolite chebi:30864 "" wikidata:Q27104161 chebi:30864;chebi:30864 inchikey:UFIVEPVSAGBUSI-REOHCLBHSA-M L-BAIBA Metabolite chebi:58655 "L form of β-aminoisobutyric acid (BAIBA)" wikidata:Q106345499 chebi:58655;chebi:58655 inchikey:QCHPKSFMDHPSNR-VKHMYHEASA-N L-methylmalonatesemialdehyde Metabolite chebi:62413 "methylmalonate semialdehyde aka 2-Methyl-3-oxopropanoic acid aka 2-methyl-3-oxopropanoate
L configuration == S-annotation in ChEBI" wikidata:Q27131865 chebi:62413;chebi:62413 inchikey:VOKUMXABRRXHAR-VKHMYHEASA-M Propionyl-CoA Metabolite chebi:57392 "" wikidata:Q27124603 chebi:57392;chebi:57392 inchikey:QAQREVBBADEHPA-IEXPHMLFSA-J UMPS-complex GeneProduct uniprot:P11172 "" ensembl:ENSG00000114491 ncbigene:7372 hgnc.symbol:UMPS uniprot:A8K5J1;uniprot:F2Z3P2;uniprot:F2Z303;uniprot:F8WDG4;uniprot:P11172;uniprot:E9PFD2 CAD-complex GeneProduct uniprot:P27708 "" ensembl:ENSG00000084774 ncbigene:790 hgnc.symbol:CAD uniprot:H7BZB3;uniprot:F8VPD4;uniprot:P27708;uniprot:H7C2E4;uniprot:H7C3Z5 RRM1 Protein uniprot:P23921 "" ensembl:ENSG00000167325 ncbigene:6240 hgnc.symbol:RRM1 uniprot:H0YCY7;uniprot:E9PJ62;uniprot:E9PL69;uniprot:E9PP77;uniprot:P23921;uniprot:E9PRY9 RRM2 Protein uniprot:P31350 "" ensembl:ENSG00000171848 ncbigene:6241 hgnc.symbol:RRM2 uniprot:A0A2R8Y837;uniprot:P31350;uniprot:A0A7P0SBL1;uniprot:A0A286YFD6 MMSDH Protein uniprot:Q02252 "" ensembl:ENSG00000119711 ncbigene:4329 hgnc.symbol:ALDH6A1 uniprot:G3V4Z4;uniprot:A0A024R6G4;uniprot:Q02252 RRM2B Protein uniprot:Q7LG56 "" ensembl:ENSG00000048392 ncbigene:50484 hgnc.symbol:RRM2B uniprot:E5RG52;uniprot:H0YAV1;uniprot:Q7LG56 TK2 Protein uniprot:O00142 "" ensembl:ENSG00000166548 ncbigene:7084 hgnc.symbol:TK2 uniprot:A0A7P0SB82;uniprot:A0A7I2V4A1;uniprot:A0A7I2V3A3;uniprot:A0A7I2YQY6;uniprot:J3QL12;uniprot:A0A7I2V3V1;uniprot:A0A7I2V471;uniprot:A0A7I2V465;uniprot:J3KSZ2;uniprot:O00142;uniprot:A0A7I2V5S4;uniprot:H3BP77;uniprot:A0A0A0MSY7;uniprot:A0A7I2V5P3;uniprot:A0A7I2V304;uniprot:A0A7I2V557;uniprot:A0A7P0PE46;uniprot:J3KS73;uniprot:J3QRP0;uniprot:A0A7I2V406;uniprot:A0A7I2V408;uniprot:A0A7P0MLU2;uniprot:A0A7I2V2L8;uniprot:H3BV57 OTC Protein uniprot:P00480 "" ensembl:ENSG00000036473 ncbigene:5009 hgnc.symbol:OTC uniprot:A0A2R8Y829;uniprot:P00480 DPD Protein uniprot:Q12882 "AKA DPYD" ensembl:ENSG00000188641 ncbigene:1806 hgnc.symbol:DPYD uniprot:Q12882 DHO Protein eccode:3.5.2.3 "" GLS2 Protein uniprot:Q9UI32 "" ensembl:ENSG00000135423 ncbigene:27165 hgnc.symbol:GLS2 uniprot:F8WBC9;uniprot:C9J3Q2;uniprot:F8WEU7;uniprot:A8K0A6;uniprot:A0A087X004;uniprot:F8WBL8;uniprot:G5E9X4;uniprot:Q9UI32;uniprot:E5G745 RRM2B Protein uniprot:Q7LG56 "" ensembl:ENSG00000048392 ncbigene:50484 hgnc.symbol:RRM2B uniprot:E5RG52;uniprot:H0YAV1;uniprot:Q7LG56 UMPH1 Protein uniprot:Q9H0P0 "aka P5N1
Enzymes and reactions added with info from https://www.omim.org/entry/606224" ensembl:ENSG00000122643 ncbigene:51251 hgnc.symbol:NT5C3A uniprot:X6RM59;uniprot:B9A035;uniprot:A0A090N7U2;uniprot:C9K084;uniprot:A0A024RA81;uniprot:F8WDR0;uniprot:Q9H0P0 UP Protein uniprot:Q9UBR1 "aka UPB1" ensembl:ENSG00000100024 ncbigene:51733 hgnc.symbol:UPB1 uniprot:A0A024R1H3;uniprot:Q9UBR1;uniprot:Q6AHZ8;uniprot:F8WC94 CPS1 Protein uniprot:P31327 "" ensembl:ENSG00000021826 ncbigene:1373 hgnc.symbol:CPS1 uniprot:P31327;uniprot:A0A024R454;uniprot:A0A669KBF2 TS Protein uniprot:P04818 "" ensembl:ENSG00000176890 ncbigene:7298 hgnc.symbol:TYMS uniprot:P04818;uniprot:Q53Y97 OPRT Protein eccode:2.4.2.10 "" OMPDC Protein eccode:4.1.1.23 "" AGXT2 Protein uniprot:Q9BYV1 "" ensembl:ENSG00000113492 ncbigene:64902 hgnc.symbol:AGXT2 uniprot:Q9BYV1 TP Protein uniprot:P19971 "" ensembl:ENSG00000025708 ncbigene:1890 hgnc.symbol:TYMP uniprot:A0A494C0A4;uniprot:C9JGI3;uniprot:A0A494BZZ4;uniprot:P19971;uniprot:A0A494C1L9;uniprot:E5KRG5;uniprot:A0A494C0L3;uniprot:A0A494C0L6;uniprot:A0A494C1N7 DHODH Protein uniprot:Q02127 "" ensembl:ENSG00000102967 ncbigene:1723 hgnc.symbol:DHODH uniprot:I3L449;uniprot:I3NI32;uniprot:J3QRQ3;uniprot:Q02127;uniprot:J3QRJ4 ACT Protein eccode:2.1.3.2 "" beta-alanine-pyruvate transaminase Protein eccode:2.6.1.18 "" CPS2 Protein eccode:6.3.5.5 "" RR Protein eccode:1.17.4.1 "" DHP Protein uniprot:Q14117 "AKA DPYS" ensembl:ENSG00000147647 ncbigene:1807 hgnc.symbol:DPYS uniprot:Q14117;uniprot:E5RG28;uniprot:H0YEV7 CPS2 Protein eccode:6.3.5.5 "" DHO Protein eccode:3.5.2.3 "" ACT Protein kegg.genes:2.1.3.2 "" OPRT Protein eccode:2.4.2.10 "" OMPDC Protein eccode:4.1.1.23 "" UMPH2 Protein uniprot:Q8TCD5 "aka NT5C
Enzymes and reactions added with info from https://www.omim.org/entry/606224" ensembl:ENSG00000125458 ncbigene:30833 hgnc.symbol:NT5C uniprot:J3KSF0;uniprot:Q8TCD5;uniprot:J3KSX6;uniprot:J3KSY6;uniprot:J3QQX8;uniprot:V9HWF3;uniprot:J3KRC4 UMPH Protein eccode:3.1.3.5 "aka P5N
Enzymes and reactions added with info from https://www.omim.org/entry/606224" UMPH1 Protein uniprot:Q9H0P0 "aka P5N1
Enzymes and reactions added with info from https://www.omim.org/entry/606224" ensembl:ENSG00000122643 ncbigene:51251 hgnc.symbol:NT5C3A uniprot:X6RM59;uniprot:B9A035;uniprot:A0A090N7U2;uniprot:C9K084;uniprot:A0A024RA81;uniprot:F8WDR0;uniprot:Q9H0P0 UMPH2 Protein uniprot:Q8TCD5 "aka NT5C
Enzymes and reactions added with info from https://www.omim.org/entry/606224" ensembl:ENSG00000125458 ncbigene:30833 hgnc.symbol:NT5C uniprot:J3KSF0;uniprot:Q8TCD5;uniprot:J3KSX6;uniprot:J3KSY6;uniprot:J3QQX8;uniprot:V9HWF3;uniprot:J3KRC4 UMPH Protein eccode:3.1.3.5 "aka P5N
Enzymes and reactions added with info from https://www.omim.org/entry/606224" RRM1 Protein uniprot:P23921 "" ensembl:ENSG00000167325 ncbigene:6240 hgnc.symbol:RRM1 uniprot:H0YCY7;uniprot:E9PJ62;uniprot:E9PL69;uniprot:E9PP77;uniprot:P23921;uniprot:E9PRY9 ABAT Protein uniprot:P80404 "" ensembl:ENSG00000183044 ncbigene:18 hgnc.symbol:ABAT uniprot:H3BNQ7;uniprot:H3BPW8;uniprot:X5D8S1;uniprot:H3BP74;uniprot:H3BMJ9;uniprot:H3BP84;uniprot:H3BRT1;uniprot:H3BRJ1;uniprot:H3BRN4;uniprot:P80404