UniProt Accession

 HEM4_HUMAN; P10746; B2RC13; D3DRF7; Q9H2T1 

Genbank Protein ID

 J03824; AF230665; AH010036; AK314896; AL360176; CH471066; CH471066; BC002573 

Genbank Nucleotide ID

 AAA60273.1; AAG36795.1; AAG36794.1; BAG37410.1; CAI12087.1; EAW49221.1; EAW49222.1; AAH02573.1 

Protein Name

 Uroporphyrinogen-III synthase 

Protein Synonyms/Alias

 UROIIIS; UROS; Hydroxymethylbilane hydrolyase [cyclizing]; Uroporphyrinogen-III cosynthase 

Gene Name

 UROS 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
7	*MKVLLLKDAKEDDC	acetylation	[1]
10	VLLLKDAKEDDCGQD	acetylation	[1]

Reference

 [1] Integrated proteomic analysis of post-translational modifications by serial enrichment.
 Mertins P, Qiao JW, Patel J, Udeshi ND, Clauser KR, Mani DR, Burgess MW, Gillette MA, Jaffe JD, Carr SA.
 Nat Methods. 2013 Jul;10(7):634-7. [PMID: 23749302] 

Functional Description

 Catalyzes cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the branch point for the various sub-pathways leading to the wide diversity of porphyrins. Porphyrins act as cofactors for a multitude of enzymes that perform a variety of processes within the cell such as methionine synthesis (vitamin B12) or oxygen transport (heme). 

Sequence Annotation

  

Keyword

 3D-structure; Complete proteome; Direct protein sequencing; Disease mutation; Heme biosynthesis; Lyase; Polymorphism; Porphyrin biosynthesis; Reference proteome. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 265 AA 

Protein Sequence

Gene Ontology

 GO:0005829; C:cytosol; NAS:UniProtKB.
 GO:0005739; C:mitochondrion; ISS:UniProtKB.
 GO:0048037; F:cofactor binding; IEA:Compara.
 GO:0004852; F:uroporphyrinogen-III synthase activity; IDA:UniProtKB.
 GO:0071418; P:cellular response to amine stimulus; IEA:Compara.
 GO:0071243; P:cellular response to arsenic-containing substance; IEA:Compara.
 GO:0006783; P:heme biosynthetic process; IDA:UniProtKB.
 GO:0006782; P:protoporphyrinogen IX biosynthetic process; IEA:UniProtKB-UniPathway.
 GO:0046677; P:response to antibiotic; IEA:Compara.
 GO:0006780; P:uroporphyrinogen III biosynthetic process; IDA:UniProtKB. 

Interpro

 IPR003754; 4pyrrol_synth_uPrphyn_synth. 

Pfam

 PF02602; HEM4 

SMART

  

PROSITE

  

PRINTS