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<Pathway xmlns="http://pathvisio.org/GPML/2013a" Name="Heme biosynthesis" Data-Source="WikiPathways" Version="WP18_r142375" Author="[Kdahlquist, MaintBot, M.Braymer, Khanspers, Mills42, Christine Chichester, Mkutmon, DeSl, Eweitz, Egonw]" Last-Modified="20260130161533" Organism="Mus musculus">
  <Comment Source="HomologyConvert">This pathway was inferred from Homo sapiens pathway [http://www.wikipathways.org/index.php?title=Pathway:WP561&amp;amp;oldid=21860 WP561(r21860)] with a 60% conversion rate.</Comment>
  <Comment Source="WikiPathways-description">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](https://en.wikipedia.org/wiki/Heme).</Comment>
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