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Literature summary extracted from

  • Yien, Y.Y.; Ducamp, S.; van der Vorm, L.N.; Kardon, J.R.; Manceau, H.; Kannengiesser, C.; Bergonia, H.A.; Kafina, M.D.; Karim, Z.; Gouya, L.; Baker, T.A.; Puy, H.; Phillips, J.D.; Nicolas, G.; Paw, B.H.
    Mutation in human CLPX elevates levels of delta-aminolevulinate synthase and protoporphyrin IX to promote erythropoietic protoporphyria (2017), Proc. Natl. Acad. Sci. USA, 114, E8045-E8052 .
    View publication on PubMedView publication on EuropePMC

Activating Compound

EC Number Activating Compound Comment Organism Structure
2.3.1.37 ClpX the mitochondrial AAA+ unfoldase ClpX participates in both heme-dependent degradation of the enzyme and its activation Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2.3.1.37 succinyl-CoA + glycine Homo sapiens
-
 5-aminolevulinate + CoA + CO2
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
2.3.1.37 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.3.1.37 erythroid cell
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.3.1.37 succinyl-CoA + glycine
-
 Homo sapiens 5-aminolevulinate + CoA + CO2
-
 ?

Synonyms

EC Number Synonyms Comment Organism
2.3.1.37 ALAS
-
 Homo sapiens
2.3.1.37 delta-aminolevulinate synthase
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
2.3.1.37 pyridoxal 5'-phosphate
-
 Homo sapiens

Expression

EC Number Organism Comment Expression
2.3.1.37 Homo sapiens the presence of low-activity mitochondrial AAA+ unfoldase CLPX increases the posttranslational stability of the enzyme, causing increased protein level up