Domain

Fumarylacetoacetase, C-terminal-related (IPR011234)

Short name: Fumarylacetoacetase_C-rel

Domain relationships

None.

Description

Fumarylacetoacetase (EC:3.7.1.2; also known as fumarylacetoacetate hydrolase or FAH) catalyses the hydrolytic cleavage of a carbon-carbon bond in fumarylacetoacetate to yield fumarate and acetoacetate as the final step in phenylalanine and tyrosine degradation [PMID: 11154690]. This is an essential metabolic function in humans, the lack of FAH causing type I tyrosinaemia, which is associated with liver and kidney abnormalities and neurological disorders [PMID: 9101289, PMID: 16602095]. The enzyme mechanism involves a catalytic metal ion, a Glu/His catalytic dyad, and a charged oxyanion hole [PMID: 10508789]. FAH folds into two domains: an N-terminal domain SH3-like beta-barrel, and a C-terminal with an unusual fold consisting of three layers of beta-sheet structures [PMID: 10508789].

This entry represents the C-terminal domain of fumarylacetoacetase, as well as other domains that share a homologous alpha/beta structure, including:

  • 5-carboxymethyl-2-hydroxymuconate delta-isomerase (CHM isomerase; EC:5.3.3.10), which catalyses the conversion of 5-carboxymethyl-2-hydroxymuconate to 5-carboxy-2-oxohept-3-enedioate [PMID: 2194841].
  • 5-oxopent-3-ene-1,2,5-tricarboxylate decarboxylase (OPET decarboxylase; EC:4.1.1.68), which catalyses the conversion of 5-oxopent-3-ene-1,2,5-tricarboxylate to 2-oxohept-3-enedioate and carbon dioxide.
  • Bifunctional enzyme HpcE (OPET decarboxylase EC:4.1.1.68/HHDD isomerase EC:5.3.3.10), which is a duplication consisting of a tandem repeat of two FAH C-terminal-like domains. This enzyme is responsible for the degradation of 4-hydroxyphenylacetate, a product of tyrosine and phenylalanine metabolism also released by lignin catabolism [PMID: 11863436].
  • 2-keto-4-pentenoate hydratase MhpD (EC:4.2.1.80; also known as 2-oxopent-4-enoate hydratase), which converts 4-hydroxy-2-oxopentanoate to 2-oxopent-4-enoate [PMID: 10537203].
  • 4-oxalocrotonate decarboxylase (4-OD; EC:4.1.1.77), which catalyses the conversion of 4-oxalocrotonate to 2-oxopent-4-enoate and carbon dioxide [PMID: 10651637].
  • 2-oxo-hepta-3-ene-1,7-dioic acid hydratase, which hydrates the double bond of 2-oxo-hepta-3-ene-1,7-dioic acid to form 4-hydroxy-2-oxo-heptane-1,7-dioic acid in the catabolism of 4-hydroxyphenylacetic acid.

GO terms

Biological Process

GO:0008152 metabolic process

Molecular Function

GO:0003824 catalytic activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
GENE3D
SUPERFAMILY
Pfam