UniProt Accession

 HEM3_HUMAN; P08397; A8K2L0; G3V1P4; G5EA58; P08396; Q16012 

Genbank Protein ID

 X04217; X04808; M95623; M95623; AK000628; AK131072; AK290275; AP003391; AP003392; CH471065; CH471065; CH471065; BC000520; BC008149; BC019323; X68018; S60381 

Genbank Nucleotide ID

 CAA27801.1; CAA28499.1; AAA60029.1; AAA60030.1; BAF82964.1; EAW67447.1; EAW67449.1; EAW67450.1; AAH00520.1; AAH08149.1; AAH19323.1; CAA48156.1; AAC60602.1 

Protein Name

 Porphobilinogen deaminase 

Protein Synonyms/Alias

 PBG-D; Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase 

Gene Name

 HMBS 

Gene Synonyms/Alias

 PBGD; UPS 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
17	TAEENSPKMRVIRVG	acetylation	[1]
70	ILDTALSKIGEKSLF	ubiquitination	[2]
74	ALSKIGEKSLFTKEL	ubiquitination	[2]
87	ELEHALEKNEVDLVV	acetylation	[3]
115	FTIGAICKRENPHDA	ubiquitination	[2]
132	FHPKFVGKTLETLPE	ubiquitination	[2]
140	TLETLPEKSVVGTSS	ubiquitination	[2]
157	RAAQLQRKFPHLEFR	ubiquitination	[2]
227	LGVEVRAKDQDILDL	ubiquitination	[2]
348	LLLSKGAKNILDVAR	ubiquitination	[2]

Reference

 [1] Regulation of cellular metabolism by protein lysine acetylation.
 Zhao S, Xu W, Jiang W, Yu W, Lin Y, Zhang T, Yao J, Zhou L, Zeng Y, Li H, Li Y, Shi J, An W, Hancock SM, He F, Qin L, Chin J, Yang P, Chen X, Lei Q, Xiong Y, Guan KL.
 Science. 2010 Feb 19;327(5968):1000-4. [PMID: 20167786]
 [2] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [3] Lysine acetylation targets protein complexes and co-regulates major cellular functions.
 Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M.
 Science. 2009 Aug 14;325(5942):834-40. [PMID: 19608861] 

Functional Description

 Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. 

Sequence Annotation

 MOD_RES 2 2 N-acetylserine.
 MOD_RES 261 261 S-(dipyrrolylmethanemethyl)cysteine.  

Keyword

 3D-structure; Acetylation; Alternative splicing; Complete proteome; Cytoplasm; Direct protein sequencing; Disease mutation; Heme biosynthesis; Porphyrin biosynthesis; Reference proteome; Transferase. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 361 AA 

Protein Sequence

Gene Ontology

 GO:0005829; C:cytosol; TAS:Reactome.
 GO:0004418; F:hydroxymethylbilane synthase activity; IDA:UniProtKB.
 GO:0006783; P:heme biosynthetic process; IC:UniProtKB.
 GO:0018160; P:peptidyl-pyrromethane cofactor linkage; IEA:InterPro.
 GO:0006782; P:protoporphyrinogen IX biosynthetic process; IEA:UniProtKB-UniPathway.
 GO:0044281; P:small molecule metabolic process; TAS:Reactome. 

Interpro

 IPR000860; 4pyrrol_synth_OHMeBilane_synth.
 IPR022419; Porphobilin_deaminase_cofac_BS.
 IPR022417; Porphobilin_deaminase_N.
 IPR022418; Porphobilinogen_deaminase_C. 

Pfam

 PF01379; Porphobil_deam
 PF03900; Porphobil_deamC 

SMART

  

PROSITE

 PS00533; PORPHOBILINOGEN_DEAM 

PRINTS

 PR00151; PORPHBDMNASE.