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- ****************************
- * Ferrochelatase signature *
- ****************************
-
- Ferrochelatase (EC 4.99.1.1) (protoheme ferro-lyase) [1,2] catalyzes the last
- step in the biosynthesis of heme, the chelation of a ferrous ion to proto-
- porphyrin IX to form protoheme.
-
- In eukaryotes ferrochelatase is a mitochondrial protein which is bound to the
- inner membrane and whose active site face the mitochondrial matrix. The mature
- form of eukaryotic ferrochelatase is composed of about 360 amino acids. In
- Escherichia coli, ferrochelatase (gene hemH) [3] is a protein of 320 amino
- acids.
-
- The Human autosomal dominant disease protoporphyria is due to the reduced
- activity of ferrochelatase.
-
- The signature pattern for this enzyme is based on a conserved region which
- contain a histidine residue which could be involved in the binding of iron.
-
- -Consensus pattern: [LIVM](3)-x-S-x-H-[GS]-[LIVM]-P-x(4)-[DEN]-x-G-D-x-Y
- -Sequences known to belong to this class detected by the pattern: ALL.
- -Other sequence(s) detected in SWISS-PROT: NONE.
- -Last update: October 1993 / Pattern and text revised.
-
- [ 1] Labbe-Bois R.
- J. Biol. Chem. 265:7278-7283(1990).
- [ 2] Brenner D.A., Frasier F.
- Proc. Natl. Acad. Sci. U.S.A. 88:849-853(1991).
- [ 3] Miyamoto K., Nakahigashi K., Nishimura K., Inokuchi H.
- J. Mol. Biol. 219:393-398(1991).
-