Heme biosynthesis (WP561)

Homo sapiens

Open in new tab Open in NDEx Open in Newt (SBGN)
Download PNG Download SVG Download JSON Download GPML Learn more about Downloads

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 , Kristina Hanspers , Caroline Miller , Andra Waagmeester , Alex Pico , Egon Willighagen , Martina Summer-Kutmon , Denise Slenter , and Eric Weitz

Activity

last edited

Discuss this pathway

Check for ongoing discussions or start your own.

Cited In

Are you planning to include this pathway in your next publication? See How to Cite and add a link here to your paper once it's online.

Organisms

Homo sapiens

Communities

Serious Request 2024 - MetaKids

Annotations

Pathway Ontology

heme biosynthetic pathway

Cell Type Ontology

bone marrow cell hematopoietic cell

Participants
Query Drugst.One

Label Type Compact URI Comment
delta-aminolevulinate Metabolite chemspider:10447539
O₂ Metabolite hmdb:HMDB0001377
porphobilinogen Metabolite cas:487-90-1
NH₃ Metabolite hmdb:HMDB0000051
H⁺ Metabolite pubchem.compound:1038
H₂O₂ Metabolite hmdb:HMDB0003125
CoA Metabolite hmdb:HMDB0001423
uroporphyrinogen III Metabolite cas:1976-85-8
CO₂ Metabolite hmdb:HMDB0001967
succinyl-CoA Metabolite hmdb:HMDB0001022
Glycine Metabolite hmdb:HMDB0000123
H₂O Metabolite hmdb:HMDB0002111 Type your comment here
coproporphyrinogen III Metabolite cas:2624-63-7
Porphobilinogen Metabolite hmdb:HMDB0000245
hydroxymethylbilane Metabolite chebi:16645
H₂O Metabolite hmdb:HMDB0002111
Fe²⁺ Metabolite pubchem.compound:27284
protoheme Metabolite cas:14875-96-8
protoporphyrin IX Metabolite cas:553-12-8
ALAS2 GeneProduct ncbigene:212
FECH GeneProduct ncbigene:2235
PPOX GeneProduct ncbigene:5498
CPOX GeneProduct ncbigene:1371
HMBS GeneProduct ncbigene:3145
ALAS1 GeneProduct ncbigene:211
ALAD GeneProduct ncbigene:210
UROS GeneProduct ncbigene:7390
UROD GeneProduct ncbigene:7389

References

  1. Heme biosynthesis pathway regulation in a model of hepatocarcinogenesis pre-initiation. Polo CF, Vazquez ES, Caballero F, Gerez E, Battle AM. Comp Biochem Physiol B. 1992 Sep;103(1):251–6. PubMed Europe PMC Scholia