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InterPro: IPR005708 Homogentisate 1,2-dioxygenase

Protein matches Help

UniProtKB

Matches:

599 proteins

Overview:	sorted by AC,	sorted by name,	of known structure,	proteins with splice variants
Detailed:	sorted by AC,	sorted by name,	of known structure	proteins with splice variants
Table:	For all matching proteins,	of known structure
Architectures
Accession List
Matches in BioMart

Accession

IPR005708 Homogentis_dOase

Type

Family

Signatures Help

Database	ID	Name	Proteins
Pfam	PF04209	HgmA	598
PANTHER	PTHR11056	Homogentis_dOase	588
TIGRFAMs	TIGR01015	hmgA	302
Signatures in BioMart

InterPro Relationships Help

Contains

IPR011051 Cupin, RmlC-type

GO Term annotation Help

Process

GO:0006559 L-phenylalanine catabolic process
GO:0006570 tyrosine metabolic process
GO:0055114 oxidation reduction

Function

GO:0004411 homogentisate 1,2-dioxygenase activity

InterPro annotation

Entry Details in BioMart

Abstract

Alkaptonuria (AKU), a rare hereditary disorder, was the first disease to be interpreted as an inborn error of metabolism. The deficiency causes homogentisic aciduria, ochronosis, and arthritis. AKU patients are deficient for homogentisate 1,2 dioxygenase (EC:1.13.11.5), the enzyme that mediates the conversion of homogentisate to maleylacetoacetate; a step in the catabolism of both tyrosine and phenylalanine.

Homogentisate + O(2) = 4-maleylacetoacetate.

Structural links Help

PDB - click here

SCOP: b.82.1.4

Database links Help

Enzyme: EC:1.13.11.5

PANDIT: PF04209

Blocks: IPB005708

Pfam Clan: CL0029.16

Taxonomic coverage Help

Overlapping InterPro entries Help

IPR005708

Numbers of overlapping proteins

Average numbers of overlapping amino acids

Example proteins Help

O09173 Homogentisate 1,2-dioxygenase

Q93099 Homogentisate 1,2-dioxygenase

Q9VKJ0 Homogentisate 1,2-dioxygenase

Q9Y041 Homogentisate 1,2-dioxygenase

Q9ZRA2 Homogentisate 1,2-dioxygenase

More proteins

Example Proteins Key

InterPro entry accession number/name and structure databases		Colour code
IPR011051	Cupin, RmlC-type
IPR005708	Homogentisate 1,2-dioxygenase
	SWISS-MODEL
	PDB Chain
	ModBase
	SCOP Domain

Publications Help

Additional Reading Open in usermanual
	Titus GP, Mueller HA, Burgner J, Rodriguez De Cordoba S, Penalva MA, Timm DE. Crystal structure of human homogentisate dioxygenase. Nat. Struct. Biol. 7 2000 542-6 [PubMed: 10876237] http://dx.doi.org/10.1038/76756

InterPro 23.1