UniProt Accession

 HEM2_HUMAN; P13716; A8K375; B2R6F2; Q16870; Q16871; Q9BVQ9 

Genbank Protein ID

 M13928; X64467; S99468; S99471; AK290490; AK312552; AY319481; AL137066; BC000977 

Genbank Nucleotide ID

 AAA51687.1; CAA45796.1; AAC60581.1; AAC60582.1; BAF83179.1; BAG35449.1; AAP72012.1; CAH70099.3; AAH00977.3 

Protein Name

 Delta-aminolevulinic acid dehydratase 

Protein Synonyms/Alias

 ALADH; Porphobilinogen synthase 

Gene Name

 ALAD 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
242	GPFRDAAKSSPAFGD	ubiquitination	[1]
281	GADMLMVKPGMPYLD	ubiquitination	[1]

Reference

 [1] 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] 

Functional Description

 Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen. 

Sequence Annotation

 ACT_SITE 199 199 Schiff-base intermediate with substrate.
 ACT_SITE 252 252 Schiff-base intermediate with substrate.
 METAL 122 122 Zinc 1; catalytic.
 METAL 124 124 Zinc 1; catalytic.
 METAL 131 131 Zinc 2.
 METAL 132 132 Zinc 1; catalytic.
 METAL 223 223 Zinc 2.
 BINDING 209 209 Substrate 1.
 BINDING 221 221 Substrate 1.
 BINDING 279 279 Substrate 2.
 BINDING 318 318 Substrate 2.  

Keyword

 3D-structure; Allosteric enzyme; Alternative splicing; Complete proteome; Direct protein sequencing; Disease mutation; Heme biosynthesis; Lyase; Metal-binding; Polymorphism; Porphyrin biosynthesis; Reference proteome; Zinc. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 330 AA 

Protein Sequence

Gene Ontology

 GO:0005829; C:cytosol; TAS:Reactome.
 GO:0032791; F:lead ion binding; IDA:UniProtKB.
 GO:0004655; F:porphobilinogen synthase activity; IDA:UniProtKB.
 GO:0008270; F:zinc ion binding; IDA:UniProtKB.
 GO:0006783; P:heme biosynthetic process; IDA:UniProtKB.
 GO:0051260; P:protein homooligomerization; IPI:UniProtKB.
 GO:0006782; P:protoporphyrinogen IX biosynthetic process; IEA:UniProtKB-UniPathway.
 GO:0044281; P:small molecule metabolic process; TAS:Reactome. 

Interpro

 IPR013785; Aldolase_TIM.
 IPR001731; Porphobilinogen_synth. 

Pfam

 PF00490; ALAD 

SMART

 SM01004; ALAD 

PROSITE

 PS00169; D_ALA_DEHYDRATASE 

PRINTS

 PR00144; DALDHYDRTASE.