Hydroxyacid oxidase 1
Details
- Name
- Hydroxyacid oxidase 1
- Synonyms
- 1.1.3.15
- Glycolate oxidase
- GOX
- GOX1
- HAOX1
- Gene Name
- HAO1
- Organism
- Humans
- Amino acid sequence
>lcl|BSEQ0012630|Hydroxyacid oxidase 1 MLPRLICINDYEQHAKSVLPKSIYDYYRSGANDEETLADNIAAFSRWKLYPRMLRNVAET DLSTSVLGQRVSMPICVGATAMQRMAHVDGELATVRACQSLGTGMMLSSWATSSIEEVAE AGPEALRWLQLYIYKDREVTKKLVRQAEKMGYKAIFVTVDTPYLGNRLDDVRNRFKLPPQ LRMKNFETSTLSFSPEENFGDDSGLAAYVAKAIDPSISWEDIKWLRRLTSLPIVAKGILR GDDAREAVKHGLNGILVSNHGARQLDGVPATIDVLPEIVEAVEGKVEVFLDGGVRKGTDV LKALALGAKAVFVGRPIVWGLAFQGEKGVQDVLEILKEEFRLAMALSGCQNVKVIDKTLV RKNPLAVSKI
- Number of residues
- 370
- Molecular Weight
- 40923.945
- Theoretical pI
- 8.29
- GO Classification
- Functions(S)-2-hydroxy-acid oxidase activity / FMN binding / glycolate oxidase activity / glyoxylate oxidase activity / long-chain-(S)-2-hydroxy-long-chain-acid oxidase activity / medium-chain-(S)-2-hydroxy-acid oxidase activity / receptor binding / very-long-chain-(S)-2-hydroxy-acid oxidase activityProcessescellular nitrogen compound metabolic process / fatty acid alpha-oxidation / glycolate catabolic process / glyoxylate metabolic process / response to oxidative stress / small molecule metabolic processComponentsperoxisomal matrix / peroxisome
- General Function
- Very-long-chain-(s)-2-hydroxy-acid oxidase activity
- Specific Function
- Has 2-hydroxyacid oxidase activity. Most active on the 2-carbon substrate glycolate, but is also active on 2-hydroxy fatty acids, with high activity towards 2-hydroxy palmitate and 2-hydroxy octanoate.
- Pfam Domain Function
- FMN_dh (PF01070)
- Transmembrane Regions
- Not Available
- Cellular Location
- Peroxisome
- Gene sequence
>lcl|BSEQ0012631|Hydroxyacid oxidase 1 (HAO1) ATGCTCCCCCGGCTAATTTGTATCAATGATTATGAACAACATGCTAAATCAGTACTTCCA AAGTCTATATATGACTATTACAGGTCTGGGGCAAATGATGAAGAAACTTTGGCTGATAAT ATTGCAGCATTTTCCAGATGGAAGCTGTATCCAAGGATGCTCCGGAATGTTGCTGAAACA GATCTGTCGACTTCTGTTTTAGGACAGAGGGTCAGCATGCCAATATGTGTGGGGGCTACG GCCATGCAGCGCATGGCTCATGTGGACGGCGAGCTTGCCACTGTGAGAGCCTGTCAGTCC CTGGGAACGGGCATGATGTTGAGTTCCTGGGCCACCTCCTCAATTGAAGAAGTGGCGGAA GCTGGTCCTGAGGCACTTCGTTGGCTGCAACTGTATATCTACAAGGACCGAGAAGTCACC AAGAAGCTAGTGCGGCAGGCAGAGAAGATGGGCTACAAGGCCATATTTGTGACAGTGGAC ACACCTTACCTGGGCAACCGTCTGGATGATGTGCGTAACAGATTCAAACTGCCGCCACAA CTCAGGATGAAAAATTTTGAAACCAGTACTTTATCATTTTCTCCTGAGGAAAATTTTGGA GACGACAGTGGACTTGCTGCATATGTGGCTAAAGCAATAGACCCATCTATCAGCTGGGAA GATATCAAATGGCTGAGAAGACTGACATCATTGCCAATTGTTGCAAAGGGCATTTTGAGA GGTGATGATGCCAGGGAGGCTGTTAAACATGGCTTGAATGGGATCTTGGTGTCGAATCAT GGGGCTCGACAACTCGATGGGGTGCCAGCCACTATTGATGTTCTGCCAGAAATTGTGGAG GCTGTGGAAGGGAAGGTGGAAGTCTTCCTGGACGGGGGTGTGCGGAAAGGCACTGATGTT CTGAAAGCTCTGGCTCTTGGCGCCAAGGCTGTGTTTGTGGGGAGACCAATCGTTTGGGGC TTAGCTTTCCAGGGGGAGAAAGGTGTTCAAGATGTCCTCGAGATACTAAAGGAAGAATTC CGGTTGGCCATGGCTCTGAGTGGGTGCCAGAATGTGAAAGTCATCGACAAGACATTGGTG AGGAAAAATCCTTTGGCCGTTTCCAAGATCTGA
- Chromosome Location
- 20
- Locus
- 20p12
- External Identifiers
Resource Link UniProtKB ID Q9UJM8 UniProtKB Entry Name HAOX1_HUMAN GenBank Protein ID 7530485 GenBank Gene ID AF244134 HGNC ID HGNC:4809 - General References
- Williams E, Cregeen D, Rumsby G: Identification and expression of a cDNA for human glycolate oxidase. Biochim Biophys Acta. 2000 Sep 7;1493(1-2):246-8. [Article]
- Jones JM, Morrell JC, Gould SJ: Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases. J Biol Chem. 2000 Apr 28;275(17):12590-7. [Article]
- Deloukas P, Matthews LH, Ashurst J, Burton J, Gilbert JG, Jones M, Stavrides G, Almeida JP, Babbage AK, Bagguley CL, Bailey J, Barlow KF, Bates KN, Beard LM, Beare DM, Beasley OP, Bird CP, Blakey SE, Bridgeman AM, Brown AJ, Buck D, Burrill W, Butler AP, Carder C, Carter NP, Chapman JC, Clamp M, Clark G, Clark LN, Clark SY, Clee CM, Clegg S, Cobley VE, Collier RE, Connor R, Corby NR, Coulson A, Coville GJ, Deadman R, Dhami P, Dunn M, Ellington AG, Frankland JA, Fraser A, French L, Garner P, Grafham DV, Griffiths C, Griffiths MN, Gwilliam R, Hall RE, Hammond S, Harley JL, Heath PD, Ho S, Holden JL, Howden PJ, Huckle E, Hunt AR, Hunt SE, Jekosch K, Johnson CM, Johnson D, Kay MP, Kimberley AM, King A, Knights A, Laird GK, Lawlor S, Lehvaslaiho MH, Leversha M, Lloyd C, Lloyd DM, Lovell JD, Marsh VL, Martin SL, McConnachie LJ, McLay K, McMurray AA, Milne S, Mistry D, Moore MJ, Mullikin JC, Nickerson T, Oliver K, Parker A, Patel R, Pearce TA, Peck AI, Phillimore BJ, Prathalingam SR, Plumb RW, Ramsay H, Rice CM, Ross MT, Scott CE, Sehra HK, Shownkeen R, Sims S, Skuce CD, Smith ML, Soderlund C, Steward CA, Sulston JE, Swann M, Sycamore N, Taylor R, Tee L, Thomas DW, Thorpe A, Tracey A, Tromans AC, Vaudin M, Wall M, Wallis JM, Whitehead SL, Whittaker P, Willey DL, Williams L, Williams SA, Wilming L, Wray PW, Hubbard T, Durbin RM, Bentley DR, Beck S, Rogers J: The DNA sequence and comparative analysis of human chromosome 20. Nature. 2001 Dec 20-27;414(6866):865-71. [Article]
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- Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. [Article]
- Murray MS, Holmes RP, Lowther WT: Active site and loop 4 movements within human glycolate oxidase: implications for substrate specificity and drug design. Biochemistry. 2008 Feb 26;47(8):2439-49. doi: 10.1021/bi701710r. Epub 2008 Jan 24. [Article]
- Bourhis JM, Vignaud C, Pietrancosta N, Gueritte F, Guenard D, Lederer F, Lindqvist Y: Structure of human glycolate oxidase in complex with the inhibitor 4-carboxy-5-[(4-chlorophenyl)sulfanyl]-1,2,3-thiadiazole. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2009 Dec 1;65(Pt 12):1246-53. doi: 10.1107/S1744309109041670. Epub 2009 Nov 27. [Article]
Drug Relations
- Drug Relations
DrugBank ID Name Drug group Pharmacological action? Actions Details DB02164 N-sulfo-flavin mononucleotide experimental unknown Details DB02279 Benzoylformic Acid experimental unknown Details DB03247 Flavin mononucleotide approved, investigational unknown Details DB06979 5-(dodecylthio)-1H-1,2,3-triazole-4-carboxylic acid experimental unknown Details DB07060 3-(INDOL-3-YL) LACTATE experimental unknown Details DB03064 3-Decyl-2,5-Dioxo-4-Hydroxy-3-Pyrroline experimental unknown Details DB07907 (2S)-2-HYDROXYOCTANOIC ACID experimental unknown Details DB04374 4-Carboxy-5-(1-Pentyl)Hexylsulfanyl-1,2,3-Triazole experimental unknown Details DB03884 Phenylpyruvic acid experimental unknown Details DB15935 Lumasiran approved, investigational yes antisense oligonucleotide Details