Heme biosynthesis (WP18)
Mus musculus
The enzymatic process that produces heme is porphyrin synthesis. The process is highly conserved across biology. In humans, this pathway serves almost exclusively to form heme. Heme biosynthesis starts with the synthesis of D-Aminolevulinic acid (dALA or δALA) from the amino acid glycine and succinyl-CoA, which is produced in the TCA cycle (Krebs cycle). This reaction is catalyzed by ALA synthase, which is a rate-limiting enzyme that is regulated by intracellular glucose and heme levels. The major organs for heme synthesis are the liver and the bone marrow. Source: Adapted from Wikipedia.
For a description of pathway objects, see the WikiPathways Legend.
Authors
Kdahlquist , Meredith Braymer , Kristina Hanspers , Caroline Miller , Christine Chichester , Martina Summer-Kutmon , Denise Slenter , Eric Weitz , and Egon WillighagenActivity
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Cited In
- Bioenergetic defects in muscle fibers of RYR1 mutant knock-in mice associated with malignant hyperthermia (2020).
- Advanced Running Performance by Genetic Predisposition in Male Dummerstorf Marathon Mice (DUhTP) Reveals Higher Sterol Regulatory Element-Binding Protein (SREBP) Related mRNA Expression in the Liver and Higher Serum Levels of Progesterone (2016).
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Organisms
Mus musculusCommunities
Annotations
Pathway Ontology
heme biosynthetic pathway| Label | Type | Compact URI | Comment |
|---|---|---|---|
| delta-aminolevulinate | Metabolite | chemspider:10447539 | |
| H⁺ | Metabolite | pubchem.compound:1038 | |
| Fe²⁺ | Metabolite | pubchem.compound:27284 | |
| O₂ | Metabolite | hmdb:HMDB0001377 | |
| Porphobilinogen | Metabolite | cas:487-90-1 | |
| NH₃ | Metabolite | hmdb:HMDB0000051 | |
| H₂O₂ | Metabolite | hmdb:HMDB0003125 | |
| CoA | Metabolite | hmdb:HMDB0001423 | |
| Uroporphyrinogen III | Metabolite | cas:1976-85-8 | |
| CO₂ | Metabolite | hmdb:HMDB0001967 | |
| H₂O | Metabolite | hmdb:HMDB0002111 | Type your comment here |
| Coproporphyrinogen III | Metabolite | cas:2624-63-7 | |
| Hydroxymethylbilane | Metabolite | chebi:16645 | |
| H₂O | Metabolite | hmdb:HMDB0002111 | |
| protoheme | Metabolite | cas:14875-96-8 | |
| Protoporphyrin IX | Metabolite | cas:553-12-8 | |
| Alas2 | GeneProduct | ncbigene:11656 | |
| Fech | GeneProduct | ncbigene:14151 | |
| Ppox | GeneProduct | ncbigene:19044 | |
| Cpox | GeneProduct | ncbigene:12892 | |
| Hmbs | GeneProduct | ncbigene:15288 | |
| Alas1 | GeneProduct | ncbigene:11655 | |
| Alad | GeneProduct | ncbigene:17025 | |
| Uros | GeneProduct | ncbigene:22276 | |
| Urod | GeneProduct | ncbigene:22275 |