Protein Information

General Information
MoonProt ID126
First appeared in release1.0
Name(s)Delta-aminolevulinic acid dehydratase Porphobilinogen synthase ALADH Gene Name: ALAD
UniProt IDP13716 (HEM2_HUMAN) Reviewed
GO termsGO: 0006778 porphyrin-containing compound metabolic process GO: 0006779 porphyrin-containing compound biosynthetic process GO: 0006782 protoporphyrinogen IX biosynthetic process GO: 0006783 heme biosynthetic process GO: 0008152 metabolic process GO: 0033014 tetrapyrrole biosynthetic process GO: 0044281 small molecule metabolic process GO: 0051260 protein homooligomerization GO:0071353 cellular response to interleukin-4 GO: 0003824 catalytic activity GO: 0004655 prophobilinogen synthase activity GO: 0008270 zinc ion binding GO: 0016829 lyase activity GO: 0032791 lead ion binding GO: 0042802 identical protein binding GO: 0046872 metal ion binding GO: 0005829 cytosol
Organisms for which functions have been demonstratedHomo sapiens
Sequence length330
FASTA sequence>sp|P13716|HEM2_HUMAN Delta-aminolevulinic acid dehydratase OS=Homo sapiens GN=ALAD PE=1 SV=1 MQPQSVLHSGYFHPLLRAWQTATTTLNASNLIYPIFVTDVPDDIQPITSLPGVARYGVKRLEEMLRPLVEEGLRCVLIFGVPSRVPKDERGSAADSEESPAIEAIHLLRKTFPNLLVACDVCLCPYTSHGHCGLLSENGAFRAEESRQRLAEVALAYAKAGCQVVAPSDMMDGRVEAIKEALMAHGLGNRVSVMSYSAKFASCFYGPFRDAAKSSPAFGDRRCYQLPPGARGLALRAVDRDVREGADMLMVKPGMPYLDIVREVKDKHPDLPLAVYHVSGEFAMLWHGAQAGAFDLKAAVLEAMTAFRRAGADIIITYYTPQLLQWLKEE
Structure Information
PDB ID1E51 1PV8
Quaternary structure
Function 1
Function description5-aminolaevulinate dehydratase, enzyme converts 2 (5-aminolevulinate) to porphobilinogen +2H20. Porphyrin-containing compound metabolism, protoporphyrin-IX biosynthesis
References for functionGibbs, P.N.B., and Jordan, M. Identification of lysine at the active site of human 5-aminolaevulinate dehydratase. (1986) Biochem J. 236, 447-451. Wetmur, J.G., Bishop, D.F., Cantelmo, C., and Desnick, R.J. Human sigma-aminolevulinate dehydratase: Nucleotide sequence of a full-length cDNA clone. (1986) Proc Natl Acad Sci. 83, 7703-7707. PMID: 3092810.
E.C. number4.2.1.24
Location of functional site(s)Active site:252,199 Binding site:279,209,318,221 Metal binding site:124,131,223,122,132
Cellular location of functionCytosol
Comments
Function 2
Function descriptionproteasome inhibitor Noncompetitively blocks proteolysis of certain protein substrates.
References for functionLi, X., Gu, M., and Etlinger, J.D. Isoaltion and characterization of a Novel Endogenous Inhibitor of the Proteasome. (1991) Biohcem. 30, 9709-9715. PMID: 1911759. Guo, G.G., Gu, M., and Etlinger, J.D. 204-kDa Proteasome Inhibitor (CF-2) is Identical to Aminolevulinic Acid Dehydratase. (1994), J Biol Chem. 269, 12399-12402. PMID: 8175643.
E.C. numberN/A
Location of functional site(s)
Cellular location of functioncytosol
Comments