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Literature summary extracted from

  • Li, X.; Knight, J.; Fargue, S.; Buchalski, B.; Guan, Z.; Inscho, E.W.; Liebow, A.; Fitzgerald, K.; Querbes, W.; Todd Lowther, W.; Holmes, R.P.
    Metabolism of (13)C5-hydroxyproline in mouse models of primary hyperoxaluria and its inhibition by RNAi therapeutics targeting liver glycolate oxidase and hydroxyproline dehydrogenase (2016), Biochim. Biophys. Acta, 1862, 233-239 .
    View publication on PubMedView publication on EuropePMC

Application

EC Number Application Comment Organism
1.1.3.15 medicine the ability of such siRNAs to reduce urinary oxalate in the mouse model suggests that this approach is promising for the treatment of primary hyperoxalurias, PH, particularly PH type I, in humans, genes HYPDH and GO appear to be the best targets for reducing the production of glyoxylate and oxalate in PH patients Mus musculus

Protein Variants

EC Number Protein Variants Comment Organism
1.1.3.15 additional information enzyme knockout by specific siRNA targeting the liver enzyme glycolate oxidase in wild-type leads to greatly decreased GO activity, 20fold increased urinary excretion of glycolate, and more than 2fold increased urinary oxalate excretion compared to controls. Treatment of mutant Agxt KO mice, that are deficient in liver alanine:glyoxylate aminotransferase, leads to significantly decreased urinary oxalate excretion, but substantially increased urinary glycolate excretion Mus musculus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.1.3.15 peroxisome
-
 Mus musculus 5777
-
1.5.5.3 mitochondrion
-
 Mus musculus 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.1.3.15 glycolate + O2 Mus musculus
-
 glyoxylate + H2O2
-
 ?
1.1.3.15 glycolate + O2 Mus musculus C57BL/6J
-
 glyoxylate + H2O2
-
 ?
1.5.5.3 trans-4-hydroxy-L-proline + a quinone Mus musculus
-
 (3R,5S)-3-hydroxy-1-pyrroline-5-carboxylate + a quinol
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.1.3.15 Mus musculus Q9WU19
-
-
1.1.3.15 Mus musculus C57BL/6J Q9WU19
-
-
1.5.5.3 Mus musculus
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.1.3.15 hepatocyte
-
 Mus musculus
-
1.1.3.15 liver
-
 Mus musculus
-
1.5.5.3 liver
-
 Mus musculus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.1.3.15 glycolate + O2
-
 Mus musculus glyoxylate + H2O2
-
 ?
1.1.3.15 glycolate + O2
-
 Mus musculus C57BL/6J glyoxylate + H2O2
-
 ?
1.5.5.3 trans-4-hydroxy-L-proline + a quinone
-
 Mus musculus (3R,5S)-3-hydroxy-1-pyrroline-5-carboxylate + a quinol
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.1.3.15 glycolate oxidase
-
 Mus musculus
1.1.3.15 HAO1
-
 Mus musculus
1.5.5.3 HPOX
-
 Mus musculus
1.5.5.3 HYPDH
-
 Mus musculus
1.5.5.3 ProDH2
-
 Mus musculus

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
1.1.3.15 7.1
-
 assay at Mus musculus

General Information

EC Number General Information Comment Organism
1.1.3.15 malfunction evaluation of the potential of siRNAs targeting the synthesis of liver glycolate oxidase or hydroxyproline dehydrogenase formulated in lipid nanoparticles, to reduce urinary oxalate excretion in Agxt KO mice. The siRNA targeting glycolate oxidase blocks a downstream step and prevents the synthesis of glyoxylate from glycolate in the liver. The ability of such siRNAs to reduce urinary oxalate in the mouse model suggests that this approach is promising for the treatment of primary hyperoxalurias, PH, particularly PH type I, in humans Mus musculus
1.5.5.3 malfunction enzyme knockdown alters glycolate and oxalate levels Mus musculus