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Aminolevulinate Delta Dehydratase (ALAD)

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ALADH; PBGS; Delta-Aminolevulinic Acid Dehydratase; Porphobilinogen Synthase

Aminolevulinate Delta Dehydratase (ALAD)
CCC C292
HGNC 395
MGI 96853
PubMed 2063868
RGD 2083
UniProt P13716
Wiki ALAD

The ALAD enzyme is composed of 8 identical subunits and catalyzes the condensation of 2 molecules of delta-aminolevulinate to form porphobilinogen (a precursor of heme, cytochromes and other hemoproteins). ALAD catalyzes the second step in the porphyrin and heme biosynthetic pathway; zinc is essential for enzymatic activity.

ALAD enzymatic activity is inhibited by lead and a defect in the ALAD structural gene can cause increased sensitivity to lead poisoning and acute hepatic porphyria. Alternatively spliced transcript variants encoding different isoforms have been identified.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPC292Hu01 Recombinant Aminolevulinate Delta Dehydratase (ALAD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAC292Hu01 Polyclonal Antibody to Aminolevulinate Delta Dehydratase (ALAD) WB; IHC; ICC; IP.
MAC292Hu22 Monoclonal Antibody to Aminolevulinate Delta Dehydratase (ALAD) WB; IHC; ICC; IP.
Assay Kits SEC292Hu ELISA Kit for Aminolevulinate Delta Dehydratase (ALAD) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Aminolevulinate Delta Dehydratase (ALAD) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Aminolevulinate Delta Dehydratase (ALAD) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Aminolevulinate Delta Dehydratase (ALAD) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Aminolevulinate Delta Dehydratase (ALAD) CLIA Kit Customized Service Offer
n/a ELISA Kit for Aminolevulinate Delta Dehydratase (ALAD) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPC292Ra01 Recombinant Aminolevulinate Delta Dehydratase (ALAD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAC292Ra01 Polyclonal Antibody to Aminolevulinate Delta Dehydratase (ALAD) WB; IHC; ICC; IP.
MAC292Ra21 Monoclonal Antibody to Aminolevulinate Delta Dehydratase (ALAD) WB; IHC; ICC; IP.
Assay Kits SEC292Ra ELISA Kit for Aminolevulinate Delta Dehydratase (ALAD) Enzyme-linked immunosorbent assay for Antigen Detection.
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  2. "RsaI polymorphism in the human delta-aminolevulinate dehydratase gene at 9q34."Nucleic Acids Res. 19:4307-4307(1991) [PubMed] [Europe PMC] [Abstract]
  3. "Cloning and expression of the defective genes from a patient with delta-aminolevulinate dehydratase porphyria."J. Clin. Invest. 89:1431-1437(1992) [PubMed] [Europe PMC] [Abstract]
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  9. "The molecular mechanism of lead inhibition of human porphobilinogen synthase."J. Biol. Chem. 276:1531-1537(2001) [PubMed] [Europe PMC] [Abstract]
  10. "Allosteric inhibition of human porphobilinogen synthase."J. Biol. Chem. 284:35807-35817(2009) [PubMed] [Europe PMC] [Abstract]
  11. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  12. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  13. "The crystal structure of human Ala-dehydratase."Submitted (OCT-1998) to the PDB data bank
  14. "Control of tetrapyrrole biosynthesis by alternate quaternary forms of porphobilinogen synthase."Nat. Struct. Biol. 10:757-763(2003) [PubMed] [Europe PMC] [Abstract]
  15. "Molecular characterization of the human delta-aminolevulinate dehydratase 2 (ALAD2) allele: implications for molecular screening of individuals for genetic susceptibility to lead poisoning."Am. J. Hum. Genet. 49:757-763(1991) [PubMed] [Europe PMC] [Abstract]
  16. "Delta-aminolevulinate dehydratase deficient porphyria: identification of the molecular lesions in a severely affected homozygote."Am. J. Hum. Genet. 49:167-174(1991) [PubMed] [Europe PMC] [Abstract]
  17. "Cloning and expression of the defective genes in delta-aminolevulinate dehydratase porphyria: compound heterozygosity in this hereditary liver disease."Trans. Assoc. Am. Physicians 105:250-259(1992) [PubMed] [Europe PMC] [Abstract]
  18. "A novel mutation of delta-aminolaevulinate dehydratase in a healthy child with 12% erythrocyte enzyme activity."Br. J. Haematol. 106:931-937(1999) [PubMed] [Europe PMC] [Abstract]
  19. "Novel molecular defects of the delta-aminolevulinate dehydratase gene in a patient with inherited acute hepatic porphyria."Hepatology 31:704-708(2000) [PubMed] [Europe PMC] [Abstract]
  20. "Dual gene defects involving delta-aminolaevulinate dehydratase and coproporphyrinogen oxidase in a porphyria patient."Br. J. Haematol. 132:237-243(2006) [PubMed] [Europe PMC] [Abstract]
  21. "ALAD porphyria is a conformational disease."Am. J. Hum. Genet. 80:329-337(2007) [PubMed] [Europe PMC] [Abstract]