Pathway Human Homo sapiens
Diseases of metabolism
R-HSA-5668914 in Reactome release 97: under Disease, with 272 genes placed in it by the mapping files and 12 child pathways in the hierarchy.
The same number in the other species
Reactome writes the mouse and rat events it infers from a human pathway under the human number with the species token. A door opens only where that species' own list in this release holds the id; neither of the other two lists holds it. Whether the event was inferred from this one is what the record says.
01The record
Reactome's own record of this pathway
What this tells you
The pathway list, the hierarchy and the genes on this page are read from the files Reactome publishes for Reactome release 97, built into this site on 2026-09-09: the pathway list, the hierarchy relationship file and the two gene mapping files. The record card is the one live read, Reactome's own record of this pathway.
Reactome's download page describes the mapping files: Mapping files link the source database identifier to the lowest level pathway diagram or subset of the pathway, all levels of the pathway hierarchy or database identifier to all reactions.
[R13] The gene list here is the all-levels mapping of NCBI Gene ids, filled from the all-levels mapping of Ensembl ids where the NCBI file has no row for a gene; each row names which file it came from by the shape of its source id. Each row carries the evidence codes the file writes for it, as written and unranked, and both where the file writes both; no page of Reactome's documentation the survey read defines the codes, so this page does not expand them. A gene id the identity files do not name is kept as the source's own id with no door rather than turned into a symbol.
On citing what a search finds, Reactome says: It should be remembered that while we strive to contain the most current and accurate data, Reactome should not be used in citations where other primary sources of information are available.
[R12] The record card prints the literature Reactome attaches to a curated pathway for that reason. The data are public domain: All data in the Reactome database and files derived from that data are licensed under the Creative Commons Public Domain Dedication (CC0). User may copy, modify, and distribute these data, even for commercial purposes, without asking for permission. Attribution is encouraged but not required.
[R10] The pathway illustrations are licensed apart, under CC BY 4.0, and none is shown here.
A gene on this list is one Reactome places in this pathway in this release, and that is all the row says: it does not say the gene is expressed in a tissue or associated with a disease, which are the other two explorers' questions, read from other sources. Reactome's papers of record are [R01] [R02].
- [R01] Ragueneau E, Gong C, Sinquin P, Sevilla C, Beavers D, Grentner A, et al. (2026). The Reactome Knowledgebase 2026. Nucleic Acids Research 54:D673-D681. PMID 41251150, doi 10.1093/nar/gkaf1223.
- [R02] Milacic M, Beavers D, Conley P, Gong C, Gillespie M, Griss J, et al. (2024). The Reactome Pathway Knowledgebase 2024. Nucleic Acids Research 52:D672-D678. PMID 37941124, doi 10.1093/nar/gkad1025.
- [R10] Reactome, reactome.org. License Agreement. https://reactome.org/license, read 2026-09-09.
- [R12] Reactome, reactome.org. Citing us. https://reactome.org/cite, read 2026-09-09.
- [R13] Reactome, reactome.org. Download. https://reactome.org/download-data, read 2026-09-09.
02The genes
Genes Reactome places in this human pathway
The mapping files place 272 genes in this human pathway; showing 1 to 100, in pages of 100, sorted by symbol for reading. The order carries no ranking.
| Gene | Authority id | Mapping file id | Evidence codes |
|---|---|---|---|
| GeneABCA3 | AuthorityHGNC:33 | Mapping file id21 NCBI file | EvidenceTAS |
| GeneABCD4 | AuthorityHGNC:68 | Mapping file id5826 NCBI file | EvidenceTAS |
| GeneACACA | AuthorityHGNC:84 | Mapping file id31 NCBI file | EvidenceTAS |
| GeneACAN | AuthorityHGNC:319 | Mapping file id176 NCBI file | EvidenceTAS |
| GeneACAT1 | AuthorityHGNC:93 | Mapping file id38 NCBI file | EvidenceTAS |
| GeneACY1 | AuthorityHGNC:177 | Mapping file id95 NCBI file | EvidenceTAS |
| GeneADA | AuthorityHGNC:186 | Mapping file id100 NCBI file | EvidenceTAS |
| GeneADAMTS1 | AuthorityHGNC:217 | Mapping file id9510 NCBI file | EvidenceTAS |
| GeneADAMTS10 | AuthorityHGNC:13201 | Mapping file id81794 NCBI file | EvidenceTAS |
| GeneADAMTS12 | AuthorityHGNC:14605 | Mapping file id81792 NCBI file | EvidenceTAS |
| GeneADAMTS13 | AuthorityHGNC:1366 | Mapping file id11093 NCBI file | EvidenceTAS |
| GeneADAMTS14 | AuthorityHGNC:14899 | Mapping file id140766 NCBI file | EvidenceTAS |
| GeneADAMTS15 | AuthorityHGNC:16305 | Mapping file id170689 NCBI file | EvidenceTAS |
| GeneADAMTS16 | AuthorityHGNC:17108 | Mapping file id170690 NCBI file | EvidenceTAS |
| GeneADAMTS17 | AuthorityHGNC:17109 | Mapping file id170691 NCBI file | EvidenceTAS |
| GeneADAMTS18 | AuthorityHGNC:17110 | Mapping file id170692 NCBI file | EvidenceTAS |
| GeneADAMTS19 | AuthorityHGNC:17111 | Mapping file id171019 NCBI file | EvidenceTAS |
| GeneADAMTS2 | AuthorityHGNC:218 | Mapping file id9509 NCBI file | EvidenceTAS |
| GeneADAMTS20 | AuthorityHGNC:17178 | Mapping file id80070 NCBI file | EvidenceTAS |
| GeneADAMTS3 | AuthorityHGNC:219 | Mapping file id9508 NCBI file | EvidenceTAS |
| GeneADAMTS4 | AuthorityHGNC:220 | Mapping file id9507 NCBI file | EvidenceTAS |
| GeneADAMTS5 | AuthorityHGNC:221 | Mapping file id11096 NCBI file | EvidenceTAS |
| GeneADAMTS6 | AuthorityHGNC:222 | Mapping file id11174 NCBI file | EvidenceTAS |
| GeneADAMTS7 | AuthorityHGNC:223 | Mapping file id11173 NCBI file | EvidenceTAS |
| GeneADAMTS8 | AuthorityHGNC:224 | Mapping file id11095 NCBI file | EvidenceTAS |
| GeneADAMTS9 | AuthorityHGNC:13202 | Mapping file id56999 NCBI file | EvidenceTAS |
| GeneADAMTSL1 | AuthorityHGNC:14632 | Mapping file id92949 NCBI file | EvidenceTAS |
| GeneADAMTSL2 | AuthorityHGNC:14631 | Mapping file id9719 NCBI file | EvidenceTAS |
| GeneADAMTSL3 | AuthorityHGNC:14633 | Mapping file id57188 NCBI file | EvidenceTAS |
| GeneADAMTSL4 | AuthorityHGNC:19706 | Mapping file id54507 NCBI file | EvidenceTAS |
| GeneADAMTSL5 | AuthorityHGNC:27912 | Mapping file id339366 NCBI file | EvidenceTAS |
| GeneAGRN | AuthorityHGNC:329 | Mapping file id375790 NCBI file | EvidenceTAS |
| GeneAHCY | AuthorityHGNC:343 | Mapping file id191 NCBI file | EvidenceTAS |
| GeneALDOB | AuthorityHGNC:417 | Mapping file id229 NCBI file | EvidenceTAS |
| GeneALG1 | AuthorityHGNC:18294 | Mapping file id56052 NCBI file | EvidenceTAS |
| GeneALG11 | AuthorityHGNC:32456 | Mapping file id440138 NCBI file | EvidenceTAS |
| GeneALG12 | AuthorityHGNC:19358 | Mapping file id79087 NCBI file | EvidenceTAS |
| GeneALG13 | AuthorityHGNC:30881 | Mapping file id79868 NCBI file | EvidenceTAS |
| GeneALG14 | AuthorityHGNC:28287 | Mapping file id199857 NCBI file | EvidenceTAS |
| GeneALG2 | AuthorityHGNC:23159 | Mapping file id85365 NCBI file | EvidenceTAS |
| GeneALG3 | AuthorityHGNC:23056 | Mapping file id10195 NCBI file | EvidenceTAS |
| GeneALG6 | AuthorityHGNC:23157 | Mapping file id29929 NCBI file | EvidenceTAS |
| GeneALG8 | AuthorityHGNC:23161 | Mapping file id79053 NCBI file | EvidenceTAS |
| GeneALG9 | AuthorityHGNC:15672 | Mapping file id79796 NCBI file | EvidenceTAS |
| GeneAMN | AuthorityHGNC:14604 | Mapping file id81693 NCBI file | EvidenceTAS |
| GeneAPRT | AuthorityHGNC:626 | Mapping file id353 NCBI file | EvidenceTAS |
| GeneARG1 | AuthorityHGNC:663 | Mapping file id383 NCBI file | EvidenceTAS |
| GeneARSB | AuthorityHGNC:714 | Mapping file id411 NCBI file | EvidenceTAS |
| GeneASL | AuthorityHGNC:746 | Mapping file id435 NCBI file | EvidenceTAS |
| GeneASS1 | AuthorityHGNC:758 | Mapping file id445 NCBI file | EvidenceTAS |
| GeneAUH | AuthorityHGNC:890 | Mapping file id549 NCBI file | EvidenceTAS |
| GeneB3GALT6 | AuthorityHGNC:17978 | Mapping file id126792 NCBI file | EvidenceTAS |
| GeneB3GAT3 | AuthorityHGNC:923 | Mapping file id26229 NCBI file | EvidenceTAS |
| GeneB3GLCT | AuthorityHGNC:20207 | Mapping file id145173 NCBI file | EvidenceTAS |
| GeneB4GALT1 | AuthorityHGNC:924 | Mapping file id2683 NCBI file | EvidenceTAS |
| GeneB4GALT7 | AuthorityHGNC:930 | Mapping file id11285 NCBI file | EvidenceTAS |
| GeneB4GAT1 | AuthorityHGNC:15685 | Mapping file id11041 NCBI file | EvidenceTAS |
| GeneBCAN | AuthorityHGNC:23059 | Mapping file id63827 NCBI file | EvidenceTAS |
| GeneBCKDHA | AuthorityHGNC:986 | Mapping file id593 NCBI file | EvidenceTAS |
| GeneBCKDHB | AuthorityHGNC:987 | Mapping file id594 NCBI file | EvidenceTAS |
| GeneBCKDK | AuthorityHGNC:16902 | Mapping file id10295 NCBI file | EvidenceTAS |
| GeneBGN | AuthorityHGNC:1044 | Mapping file id633 NCBI file | EvidenceTAS |
| GeneBTD | AuthorityHGNC:1122 | Mapping file id686 NCBI file | EvidenceTAS |
| GeneC1GALT1 | AuthorityHGNC:24337 | Mapping file id56913 NCBI file | EvidenceTAS |
| GeneC1GALT1C1 | AuthorityHGNC:24338 | Mapping file id29071 NCBI file | EvidenceTAS |
| GeneCBLIF | AuthorityHGNC:4268 | Mapping file id2694 NCBI file | EvidenceTAS |
| GeneCD320 | AuthorityHGNC:16692 | Mapping file id51293 NCBI file | EvidenceTAS |
| GeneCFP | AuthorityHGNC:8864 | Mapping file id5199 NCBI file | EvidenceTAS |
| GeneCHST14 | AuthorityHGNC:24464 | Mapping file id113189 NCBI file | EvidenceTAS |
| GeneCHST3 | AuthorityHGNC:1971 | Mapping file id9469 NCBI file | EvidenceTAS |
| GeneCHST6 | AuthorityHGNC:6938 | Mapping file id4166 NCBI file | EvidenceTAS |
| GeneCHSY1 | AuthorityHGNC:17198 | Mapping file id22856 NCBI file | EvidenceTAS |
| GeneCPS1 | AuthorityHGNC:2323 | Mapping file id1373 NCBI file | EvidenceTAS |
| GeneCSF2RA | AuthorityHGNC:2435 | Mapping file id1438 NCBI file | EvidenceTAS |
| GeneCSF2RB | AuthorityHGNC:2436 | Mapping file id1439 NCBI file | EvidenceTAS |
| GeneCSPG4 | AuthorityHGNC:2466 | Mapping file id1464 NCBI file | EvidenceTAS |
| GeneCSPG5 | AuthorityHGNC:2467 | Mapping file id10675 NCBI file | EvidenceTAS |
| GeneCTSA | AuthorityHGNC:9251 | Mapping file id5476 NCBI file | EvidenceTAS |
| GeneCUBN | AuthorityHGNC:2548 | Mapping file id8029 NCBI file | EvidenceTAS |
| GeneCYP11A1 | AuthorityHGNC:2590 | Mapping file id1583 NCBI file | EvidenceTAS |
| GeneCYP11B1 | AuthorityHGNC:2591 | Mapping file id1584 NCBI file | EvidenceTAS |
| GeneCYP11B2 | AuthorityHGNC:2592 | Mapping file id1585 NCBI file | EvidenceTAS |
| GeneCYP17A1 | AuthorityHGNC:2593 | Mapping file id1586 NCBI file | EvidenceTAS |
| GeneCYP19A1 | AuthorityHGNC:2594 | Mapping file id1588 NCBI file | EvidenceTAS |
| GeneCYP1B1 | AuthorityHGNC:2597 | Mapping file id1545 NCBI file | EvidenceTAS |
| GeneCYP21A2 | AuthorityHGNC:2600 | Mapping file id1589 NCBI file | EvidenceTAS |
| GeneCYP24A1 | AuthorityHGNC:2602 | Mapping file id1591 NCBI file | EvidenceTAS |
| GeneCYP26B1 | AuthorityHGNC:20581 | Mapping file id56603 NCBI file | EvidenceTAS |
| GeneCYP26C1 | AuthorityHGNC:20577 | Mapping file id340665 NCBI file | EvidenceTAS |
| GeneCYP27A1 | AuthorityHGNC:2605 | Mapping file id1593 NCBI file | EvidenceTAS |
| GeneCYP27B1 | AuthorityHGNC:2606 | Mapping file id1594 NCBI file | EvidenceTAS |
| GeneCYP2R1 | AuthorityHGNC:20580 | Mapping file id120227 NCBI file | EvidenceTAS |
| GeneCYP2U1 | AuthorityHGNC:20582 | Mapping file id113612 NCBI file | EvidenceTAS |
| GeneCYP4F22 | AuthorityHGNC:26820 | Mapping file id126410 NCBI file | EvidenceTAS |
| GeneCYP7B1 | AuthorityHGNC:2652 | Mapping file id9420 NCBI file | EvidenceTAS |
| GeneDAG1 | AuthorityHGNC:2666 | Mapping file id1605 NCBI file | EvidenceTAS |
| GeneDBT | AuthorityHGNC:2698 | Mapping file id1629 NCBI file | EvidenceTAS |
| GeneDCN | AuthorityHGNC:2705 | Mapping file id1634 NCBI file | EvidenceTAS |
| GeneDCXR | AuthorityHGNC:18985 | Mapping file id51181 NCBI file | EvidenceTAS |
| GeneDHDDS | AuthorityHGNC:20603 | Mapping file id79947 NCBI file | EvidenceTAS |
Evidence codes on this page: TAS, as the mapping file writes them; a row carrying two was written twice by the file, once under each. The mapping file id says which file placed the gene: an NCBI Gene id from NCBI2Reactome_All_Levels.txt, an Ensembl gene id from Ensembl2Reactome_All_Levels.txt. The two files can disagree about a code, so a row's codes are the one file's view. Measured by this site's build over this release on 2026-09-09: of the 138,908 human pathway-gene pairs both files place, 207 (0.149 per cent, over 47 genes) carry TAS in the Ensembl file where the NCBI rows carry IEA alone.
- Reactome mapping files, the human rows for this pathway · Reactome release 97 · read · Reactome downloads"NCBI2Reactome_All_Levels.txt" and "Ensembl2Reactome_All_Levels.txt", Reactome, release 97, https://reactome.org/download-data/ (date of access 2026-09-09). Data CC0; attribution encouraged.
03The hierarchy
Parents and children in this release's hierarchy
Children
- Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarateR-HSA-29780921 genes
- Defective ACTH causes obesity and POMCDR-HSA-55790312 genes
- Defects in vitamin and cofactor metabolismR-HSA-329648222 genes
- Diseases associated with surfactant metabolismR-HSA-568761310 genes
- Diseases of branched-chain amino acid catabolismR-HSA-986511813 genes
- Diseases of carbohydrate metabolismR-HSA-566308434 genes
- Diseases of glycosylationR-HSA-3781865146 genes
- Diseases of mitochondrial beta oxidationR-HSA-97597742 genes
- Diseases of nucleotide metabolismR-HSA-97358044 genes
- Diseases of the urea cycleR-HSA-99556988 genes
- Metabolic disorders of biological oxidation enzymesR-HSA-557902934 genes
- PhenylketonuriaR-HSA-21604561 genes
Reactome's hierarchy is a graph rather than a tree: a pathway can sit under more than one parent, and the gene counts are each pathway's own placements at every level under it, so a parent's count is not the sum of its children's.
- Reactome, the human pathway list and hierarchy relationship file · Reactome release 97 · read · Reactome downloads"ReactomePathways.txt" and "ReactomePathwaysRelation.txt", Reactome, release 97, https://reactome.org/download-data/ (date of access 2026-09-09). Data CC0; attribution encouraged.