View source for Hydroxyacid oxidase (glycolate oxidase) 1

{{Infobox_gene}}

'''Hydroxyacid oxidase (glycolate oxidase) 1''' is a [[protein]] that in humans is encoded by the HAO1 [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: Hydroxyacid oxidase (glycolate oxidase) 1
| url = https://www.ncbi.nlm.nih.gov/gene/54363 }}</ref>

==Function==

This gene is one of three related genes that have [[2-hydroxyacid]] [[oxidase]] activity yet differ in encoded protein amino acid sequence, tissue expression and substrate preference. Subcellular location of the encoded protein is the [[peroxisome]]. Specifically, this gene is expressed primarily in liver and pancreas and the encoded protein is most active on [[glycolate]], a two-carbon substrate. Glycolate oxidase oxidizes glycolic acid to glyoxylate, and can also oxidize glyoxylate into [[oxalate]]. These reactions are central to the toxicity of [[ethylene glycol poisoning]].<ref>{{Cite journal|last=Woolf|first=Alan D.|last2=Wynshaw-Boris|first2=Anthony|last3=Rinaldo|first3=Piero|last4=Levy|first4=Harvey L.|date=March 1992|title=Intentional infantile ethylene glycol poisoning presenting as an inherited metabolic disorder|url=http://dx.doi.org/10.1016/s0022-3476(05)80910-2|journal=The Journal of Pediatrics|volume=120|issue=3|pages=421–424|doi=10.1016/s0022-3476(05)80910-2|issn=0022-3476}}</ref>

The protein is also active on 2-hydroxy fatty acids. The transcript detected at high levels in pancreas may represent an alternatively spliced form or the use of a multiple near-consensus upstream [[polyadenylation]] site.<ref name="entrez" />

== References ==

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== Further reading ==

{{refbegin | 2}}
*{{cite journal |vauthors=Bourhis JM, Vignaud C, Pietrancosta N, Guéritte F, Guénard D, Lederer F, Lindqvist Y | title = Structure of human glycolate oxidase in complex with the inhibitor 4-carboxy-5-(4-chlorophenyl)sulfanyl-1,2,3-thiadiazole | journal = Acta Crystallographica Section F | volume = 65 | issue = Pt 12 | pages = 1246–53 | year = 2009 | pmid = 20054120 | pmc = 2802872 | doi = 10.1107/S1744309109041670 }}
*{{cite journal |vauthors=Jones JM, Morrell JC, Gould SJ | title = Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases | journal = The Journal of Biological Chemistry | volume = 275 | issue = 17 | pages = 12590–7 | year = 2000 | pmid = 10777549 | doi=10.1074/jbc.275.17.12590}}
*{{cite journal |vauthors=Murray MS, Holmes RP, Lowther WT | title = Active site and loop 4 movements within human glycolate oxidase: Implications for substrate specificity and drug design | journal = Biochemistry | volume = 47 | issue = 8 | pages = 2439–49 | year = 2008 | pmid = 18215067 | pmc = 2683390 | doi = 10.1021/bi701710r }}
*{{cite journal |vauthors=Lan S, Wang H, Jiang H, Mao H, Liu X, Zhang X, Hu Y, Xiang L, Yuan Z | title = Direct interaction between &alpha;-actinin and hepatitis C virus NS5B | journal = FEBS Letters | volume = 554 | issue = 3 | pages = 289–94 | year = 2003 | pmid = 14623081 | pmc =  | doi = 10.1016/S0014-5793(03)01163-3 }}
*{{cite journal |vauthors=Williams E, Cregeen D, Rumsby G | title = Identification and expression of a cDNA for human glycolate oxidase | journal = Biochimica et Biophysica Acta | volume = 1493 | issue = 1-2 | pages = 246–8 | year = 2000 | pmid = 10978532 | doi=10.1016/s0167-4781(00)00161-5}}
*{{cite journal |vauthors=Pennati A, Gadda G | title = Stabilization of an intermediate in the oxidative half-reaction of human liver glycolate oxidase | journal = Biochemistry | volume = 50 | issue = 1 | pages = 1–3 | year = 2011 | pmid = 21141873 | doi = 10.1021/bi101387w }}
*{{cite journal |vauthors=Vignaud C, Pietrancosta N, Williams EL, Rumsby G, Lederer F | title = Purification and characterization of recombinant human liver glycolate oxidase | journal = Archives of Biochemistry and Biophysics | volume = 465 | issue = 2 | pages = 410–6 | year = 2007 | pmid = 17669354 | doi = 10.1016/j.abb.2007.06.021 }}
*{{cite journal | author = Danpure CJ | title = Primary hyperoxaluria: From gene defects to designer drugs? | journal = Nephrology Dialysis Transplantation | volume = 20 | issue = 8 | pages = 1525–1529 | year = 2005 | pmid = 15956068 | pmc =  | doi = 10.1093/ndt/gfh923 }}
*{{cite journal |vauthors=Han S, Lee KM, Park SK, Lee JE, Ahn HS, Shin HY, Kang HJ, Koo HH, Seo JJ, Choi JE, Ahn YO, Kang D | title = Genome-wide association study of childhood acute lymphoblastic leukemia in Korea | journal = Leukemia Research | volume = 34 | issue = 10 | pages = 1271–4 | year = 2010 | pmid = 20189245 | doi = 10.1016/j.leukres.2010.02.001 }}

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