General Information of Protein (ID: PRT00309)
Name 3-hydroxyanthranilate oxygenase (HAAO)
Synonyms   Click to Show/Hide Synonyms of This Protein
3-hydroxyanthranilate oxygenase; 3-HAO; h3HAO; 3-hydroxyanthranilic acid dioxygenase; HAD; HAAO
Gene Name HAAO Gene ID
23498
UniProt ID
P46952
Family Oxidoreductases (EC 1)
EC Number   EC: 1.13.11.6  (Click to Show/Hide the Complete EC Tree)
Oxidoreductase
Oxygen single donor oxidoreductase
Oxygen single donor oxidoreductase
EC: 1.13.11.6
  Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein
Sequence
MERRLGVRAWVKENRGSFQPPVCNKLMHQEQLKVMFIGGPNTRKDYHIEEGEEVFYQLEG
DMVLRVLEQGKHRDVVIRQGEIFLLPARVPHSPQRFANTVGLVVERRRLETELDGLRYYV
GDTMDVLFEKWFYCKDLGTQLAPIIQEFFSSEQYRTGKPIPDQLLKEPPFPLSTRSIMEP
MSLDAWLDSHHRELQAGTPLSLFGDTYETQVIAYGQGSSEGLRQNVDVWLWQLEGSSVVT
MGGRRLSLAPDDSLLVLAGTSYAWERTQGSVALSVTQDPACKKPLG
Structure
2QNK ; 5TK5 ; 5TKQ
Function Catalyzes the oxidative ring opening of 3-hydroxyanthranilate to 2-amino-3-carboxymuconate semialdehyde, which spontaneously cyclizes to quinolinate.
Regulatory Network
Full List of Metabolite(s) Regulated by This Protein
      Nucleosides, nucleotides, and analogues
            NAD Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.558G-A) of HAAO
                      Induced Change NAD concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.558G-A) of HAAO leads to the decrease of NAD levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.483dupT) of HAAO
                      Induced Change NAD concentration: decrease (FC= 0.33)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.483dupT) of HAAO leads to the decrease of NAD levels compared with control group.
      Benzenoids
            3-Hydroxyanthranilic acid Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.483dupT) of HAAO
                      Induced Change 3-Hydroxyanthranilic acid concentration: increase (FC = 63.6)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.483dupT) of HAAO leads to the increase of 3-hydroxyanthranilic acid levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.558G-A) of HAAO
                      Induced Change 3-Hydroxyanthranilic acid concentration: increase (FC = 384.9)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.558G-A) of HAAO leads to the increase of 3-hydroxyanthranilic acid levels compared with control group.
      Organic oxygen compounds
            L-3-Hydroxykynurenine Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.483dupT) of HAAO
                      Induced Change L-3-Hydroxykynurenine concentration: increase (FC = 1.9)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.483dupT) of HAAO leads to the increase of L-3-hydroxykynurenine levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.558G-A) of HAAO
                      Induced Change L-3-Hydroxykynurenine concentration: increase (FC = 3.4)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.558G-A) of HAAO leads to the increase of L-3-hydroxykynurenine levels compared with control group.
            L-Kynurenine Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.483dupT) of HAAO
                      Induced Change L-Kynurenine concentration: increase (FC = 1.33)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.483dupT) of HAAO leads to the increase of L-kynurenine levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.558G-A) of HAAO
                      Induced Change L-Kynurenine concentration: increase (FC = 1.2)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.558G-A) of HAAO leads to the increase of L-kynurenine levels compared with control group.
      Organoheterocyclic compounds
            Picolinic acid Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.483dupT) of HAAO
                      Induced Change Picolinic acid concentration: decrease (FC= 0.45)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.483dupT) of HAAO leads to the decrease of picolinic acid levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.558G-A) of HAAO
                      Induced Change Picolinic acid concentration: increase (FC = 1.1)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.558G-A) of HAAO leads to the increase of picolinic acid levels compared with control group.
            Quinolinic acid Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.483dupT) of HAAO
                      Induced Change Quinolinic acid concentration: decrease (FC= 0.45)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.483dupT) of HAAO leads to the decrease of quinolinic acid levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.558G-A) of HAAO
                      Induced Change Quinolinic acid concentration: decrease (FC= 0.26)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.558G-A) of HAAO leads to the decrease of quinolinic acid levels compared with control group.
            Tryptophan Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.483dupT) of HAAO
                      Induced Change Tryptophan concentration: increase (FC = 1.3)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.483dupT) of HAAO leads to the increase of tryptophan levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (c.558G-A) of HAAO
                      Induced Change Tryptophan concentration: increase (FC = 1.1)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hereditary methemoglobinemia [ICD-11: 3A92]
                      Details It is reported that mutation (c.558G-A) of HAAO leads to the increase of tryptophan levels compared with control group.
References
1 NAD Deficiency, Congenital Malformations, and Niacin Supplementation. N Engl J Med. 2017 Aug 10;377(6):544-552.

If you find any error in data or bug in web service, please kindly report it to Dr. Zhang and Dr. Mou.