Gene name: HAO1

Uniprot entry:

Q9UJM8

Protein names:

2-Hydroxyacid oxidase 1 (HAOX1) (EC 1.1.3.15) (Glycolate oxidase) (GO) (GOX) (Glyoxylate oxidase) (EC 1.2.3.5)

Protein sequence:

1_MLPRL 6_ ICIND 11_ YEQHA 16_ KSVLP 21_ KSIYD 26_ YYRSG 31_ ANDEE 36_ TLADN 41_ IAAFS 46_ RWKLY 51_ PRMLR 56_ NVAET 61_ DLSTS 66_ VLGQR 71_ VSMPI 76_ CVGAT 81_ AMQRM 86_ AHVDG 91_ ELATV 96_ RACQS 101_ LGTGM 106_ MLSSW 111_ ATSSI 116_ EEVAE 121_ AGPEA 126_ LRWLQ 131_ LYIYK 136_ DREVT 141_ KKLVR 146_ QAEKM 151_ GYKAI 156_ FVTVD 161_ TPYLG 166_ NRLDD 171_ VRNRF 176_ KLPPQ 181_ LRMKN 186_ FETST 191_ LSFSP 196_ EENFG 201_ DDSGL 206_ AAYVA 211_ KAIDP 216_ SISWE 221_ DIKWL 226_ RRLTS 231_ LPIVA 236_ KGILR 241_ GDDAR 246_ EAVKH 251_ GLNGI 256_ LVSNH 261_ GARQL 266_ DGVPA 271_ TIDVL 276_ PEIVE 281_ AVEGK 286_ VEVFL 291_ DGGVR 296_ KGTDV 301_ LKALA 306_ LGAKA 311_ VFVGR 316_ PIVWG 321_ LAFQG 326_ EKGVQ 331_ DVLEI 336_ LKEEF 341_ RLAMA 346_ LSGCQ 351_ NVKVI 356_ DKTLV 361_RKNPL

Protein annotations

Protein functions:

1: Broad substrate specificity (S)-2-hydroxy-acid oxidase that preferentially oxidizes glycolate (PubMed:10777549, PubMed:10978532, PubMed:17669354, PubMed:18215067). The glyoxylate produced by the oxidation of glycolate can then be utilized by alanine-glyoxylate aminotransferase for the peroxisomal synthesis of glycine; this pathway appears to be an important step for the detoxification of glyoxylate which, if allowed to accumulate, may be metabolized to oxalate with formation of kidney stones (PubMed:10978532, PubMed:17669354). Can also catalyze the oxidation of glyoxylate, and long chain hydroxyacids such as 2-hydroxyhexadecanoate and 2-hydroxyoctanoate, albeit with much lower catalytic efficiency (PubMed:10777549, PubMed:17669354, PubMed:18215067). Active in vitro with the artificial electron acceptor 2,6-dichlorophenolindophenol (DCIP), but O2 is believed to be the physiological electron acceptor, leading to the production of H2O2 (PubMed:10777549, PubMed:10978532, PubMed:17669354, PubMed:18215067). Is not active on L-lactate and 2-hydroxybutanoate (PubMed:10777549)