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Literature summary for 1.13.11.5 extracted from

  • Titus, G.P.; Mueller, H.A.; Burgner, J.; Rodriguez de Cordoba, S.; Penalva, M.A.; Timm, D.E.
    Crystal structure of human homogentisate dioxygenase (2000), Nat. Struct. Biol., 7, 542-546.
    View publication on PubMed

Application

Application Comment Organism
medicine
-
Mus musculus
medicine HGO deficiency causes alkaptonuria, inherited as a recessive Mendelian trait, HGO inhibitors may be useful in the treatment of hereditary tyrosinemia type I, HT1 Homo sapiens

Crystallization (Commentary)

Crystallization (Comment) Organism
crystal structures of apo-HGO and HGO containing an iron Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information
-
Mus musculus
additional information 20 missence mutations in HGO from alkaptonuria patients Homo sapiens

Metals/Ions

Metals/Ions Comment Organism Structure
Fe2+
-
Mus musculus
Fe2+
-
Bos taurus
Fe2+ contains a nonheme Fe2+ at the active site, that is coordinated near the interface between subunits in the HGO trimer by Glu-341, His-335 and His-371 Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
150000
-
-
Mus musculus
254000
-
-
Bos taurus
424000 478000 equilibrium sedimentation studies Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
homogentisate + O2 Mus musculus
-
4-maleylacetoacetate
-
?
homogentisate + O2 Homo sapiens enzyme cleaves the aromatic ring during the metabolic degradation of phenylalanine and tyrosine 4-maleylacetoacetate
-
?

Organism

Organism UniProt Comment Textmining
Bos taurus
-
-
-
Homo sapiens
-
-
-
Mus musculus
-
-
-

Purification (Commentary)

Purification (Comment) Organism
-
Homo sapiens

Reaction

Reaction Comment Organism Reaction ID
homogentisate + O2 = 4-maleylacetoacetate catalytic mechanism Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
homogentisate + O2
-
Mus musculus 4-maleylacetoacetate
-
?
homogentisate + O2 utilizes a nonheme iron to incorporate both atoms of molecular oxygen into homogentisate, identical with 2,5-dihydroxyphenylacetate Homo sapiens 4-maleylacetoacetate
-
?
homogentisate + O2 enzyme cleaves the aromatic ring during the metabolic degradation of phenylalanine and tyrosine Homo sapiens 4-maleylacetoacetate
-
?
additional information enzyme structure, conformation of the active site, enzyme contains a 280-residue N-terminal domain and a 140-residue C-terminal domain, associated as a hexamer arranged as a dimer of trimers Homo sapiens ?
-
?

Subunits

Subunits Comment Organism
hexamer hexamer arranged as a dimer of trimers, subunit structure, functional importance of the hexamer in vivo Homo sapiens