Hmdb loader
Identification
HMDB Protein ID HMDBP00842
Secondary Accession Numbers
  • 6123
Name Homogentisate 1,2-dioxygenase
Synonyms
  1. Homogentisate oxygenase
  2. Homogentisic acid oxidase
  3. Homogentisicase
Gene Name HGD
Protein Type Unknown
Biological Properties
General Function Involved in homogentisate 1,2-dioxygenase activity
Specific Function Not Available
Pathways
  • Alkaptonuria
  • Disulfiram Action Pathway
  • Dopamine beta-hydroxylase deficiency
  • Hawkinsinuria
  • L-phenylalanine degradation
  • Monoamine oxidase-a deficiency (MAO-A)
  • Phenylalanine and Tyrosine Metabolism
  • Phenylketonuria
  • Tyrosine metabolism
  • Tyrosine metabolism
  • Tyrosinemia Type 2 (or Richner-Hanhart syndrome)
  • Tyrosinemia Type 3 (TYRO3)
  • Tyrosinemia Type I
  • Tyrosinemia, transient, of the newborn
Reactions
Homogentisic acid + Oxygen → Maleylacetoacetic acid details
GO Classification
Biological Process
cellular nitrogen compound metabolic process
L-phenylalanine catabolic process
tyrosine catabolic process
Cellular Component
cytosol
Function
catalytic activity
oxidoreductase activity, acting on single donors with incorporation of molecular oxygen
oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen
oxidoreductase activity
homogentisate 1,2-dioxygenase activity
Molecular Function
metal ion binding
homogentisate 1,2-dioxygenase activity
Process
l-phenylalanine metabolic process
l-phenylalanine catabolic process
metabolic process
cellular metabolic process
oxidation reduction
cellular amino acid and derivative metabolic process
cellular amino acid metabolic process
tyrosine metabolic process
aromatic amino acid family metabolic process
Cellular Location Not Available
Gene Properties
Chromosome Location 3
Locus 3q13.33
SNPs HGD
Gene Sequence
>1338 bp
ATGGCTGAGTTAAAGTACATTTCTGGATTTGGGAATGAGTGTTCTTCAGAGGATCCTCGC
TGCCCAGGTTCCCTGCCAGAAGGACAGAATAATCCTCAGGTCTGCCCCTACAATCTCTAT
GCTGAGCAGCTCTCAGGATCGGCTTTCACTTGTCCACGGAGCACCAATAAGAGAAGCTGG
CTGTATAGGATTCTACCTTCAGTTTCTCACAAGCCCTTTGAATCCATTGACGAAGGCCAA
GTCACTCACAACTGGGATGAAGTTGATCCTGATCCTAACCAGCTTAGATGGAAACCATTT
GAGATTCCAAAAGCATCTCAGAAGAAAGTAGACTTTGTGAGTGGCCTGCATACCTTGTGT
GGAGCTGGAGACATAAAGTCTAACAATGGGCTTGCTATCCACATTTTCCTCTGCAATACC
TCCATGGAGAACAGATGCTTTTACAATTCAGATGGGGACTTCTTGATTGTTCCGCAGAAA
GGGAACCTTCTCATTTACACCGAGTTTGGCAAGATGCTTGTACAGCCCAATGAGATCTGC
GTCATTCAGAGAGGAATGCGGTTCAGCATAGATGTCTTTGAGGAGACCAGGGGCTACATC
TTGGAGGTCTATGGTGTCCACTTTGAGTTACCTGACCTTGGACCAATTGGGGCCAATGGC
TTGGCCAATCCTCGTGATTTCTTGATACCCATTGCCTGGTATGAGGATCGCCAAGTACCA
GGTGGTTACACGGTCATTAATAAATACCAGGGCAAGCTGTTTGCTGCCAAACAGGATGTC
TCCCCGTTCAATGTTGTGGCCTGGCACGGGAATTATACACCCTACAAGTACAACCTGAAG
AATTTCATGGTTATCAACTCAGTGGCCTTTGACCATGCAGACCCATCCATTTTCACAGTA
TTGACTGCTAAGTCTGTCCGCCCTGGAGTGGCCATTGCTGATTTTGTCATCTTCCCACCT
CGATGGGGGGTTGCTGATAAGACCTTCAGGCCTCCTTATTACCATAGGAACTGCATGAGT
GAGTTCATGGGACTCATCCGAGGTCACTATGAGGCAAAGCAAGGTGGGTTCCTGCCAGGG
GGAGGGAGTCTACACAGCACAATGACCCCCCATGGACCTGATGCTGACTGCTTTGAGAAG
GCCAGCAAGGTCAAGCTGGCACCTGAGAGGATTGCCGATGGCACCATGGCATTTATGTTT
GAATCATCTTTAAGTCTGGCGGTCACAAAGTGGGGACTCAAGGCCTCCAGGTGTTTGGAT
GAGAACTACCACAAGTGCTGGGAGCCACTCAAGAGCCACTTCACTCCCAACTCCAGGAAC
CCAGCAGAACCTAATTGA
Protein Properties
Number of Residues 445
Molecular Weight 49963.41
Theoretical pI 6.962
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Homogentisate 1,2-dioxygenase
MAELKYISGFGNECSSEDPRCPGSLPEGQNNPQVCPYNLYAEQLSGSAFTCPRSTNKRSW
LYRILPSVSHKPFESIDEGQVTHNWDEVDPDPNQLRWKPFEIPKASQKKVDFVSGLHTLC
GAGDIKSNNGLAIHIFLCNTSMENRCFYNSDGDFLIVPQKGNLLIYTEFGKMLVQPNEIC
VIQRGMRFSIDVFEETRGYILEVYGVHFELPDLGPIGANGLANPRDFLIPIAWYEDRQVP
GGYTVINKYQGKLFAAKQDVSPFNVVAWHGNYTPYKYNLKNFMVINSVAFDHADPSIFTV
LTAKSVRPGVAIADFVIFPPRWGVADKTFRPPYYHRNCMSEFMGLIRGHYEAKQGGFLPG
GGSLHSTMTPHGPDADCFEKASKVKLAPERIADGTMAFMFESSLSLAVTKWGLKASRCLD
ENYHKCWEPLKSHFTPNSRNPAEPN
GenBank ID Protein 115527117
UniProtKB/Swiss-Prot ID Q93099
UniProtKB/Swiss-Prot Entry Name HGD_HUMAN
PDB IDs
GenBank Gene ID NM_000187.3
GeneCard ID HGD
GenAtlas ID HGD
HGNC ID HGNC:4892
References
General References
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