title: Enzymological characterization of a putative canine analogue of primary hyperoxaluria type 1.
abstract: This paper concerns an enzymological investigation into a putative canine analogue of the human autosomal recessive disease primary hyperoxaluria type 1 (alanine:glyoxylate/serine:pyruvate aminotransferase deficiency). The liver and kidney activities of alanine:glyoxylate aminotransferase and serine:pyruvate aminotransferase in two Tibetan Spaniel pups with familial oxalate nephropathy were markedly reduced when compared with a variety of controls. There were no obvious deficiencies in a number of other enzymes including D-glycerate dehydrogenase/glyoxylate reductase which have been shown previously to be deficient in primary hyperoxaluria type 2. Immunoblotting of liver and kidney homogenates from oxalotic dogs also demonstrated a severe deficiency of immunoreactive alanine:glyoxylate aminotransferase. The developmental expression of alanine:glyoxylate/serine:pyruvate aminotransferase was studied in the livers and kidneys of control dogs. In the liver, enzyme activity and immunoreactive protein were virtually undetectable at 1 day old, but then increased to reach a plateau between 4 and 12 weeks. During this period the activity was similar to that found in normal human liver. The enzyme activities and the levels of immunoreactive protein in the kidneys were more erratic, but they appeared to increase up to 8 weeks and then decrease, so that by 36 weeks the levels were similar to those found at 1 day. The data presented in this paper suggest that these oxalotic dogs have a genetic condition that is analogous, at least enzymologically, to the human disease primary hyperoxaluria type 1.
authors.affiliation: Biochemical Genetics Research Group, Clinical Research Centre, Middlesex, U.K.
authors.forename: C J
authors.initials: CJ
authors.lastname: Danpure
authors.forename: P R
authors.initials: PR
authors.lastname: Jennings
authors.forename: J H
authors.initials: JH
authors.lastname: Jansen
mesh_terms: D000410:Alanine Transaminase / Q000172:deficiency / Q000276:immunology / Q000378:metabolism; D000818:Animals; D001219:Aspartate Aminotransferases / Q000378:metabolism; D015153:Blotting, Western; D003605:D-Amino-Acid Oxidase / Q000378:metabolism; D004283:Dog Diseases / Q000201:enzymology*; D004285:Dogs; D006959:Hyperoxaluria / Q000201:enzymology / Q000662:veterinary*; D007668:Kidney / Q000201:enzymology; D007770:L-Lactate Dehydrogenase / Q000378:metabolism; D008099:Liver / Q000201:enzymology; D000637:Transaminases / Q000378:metabolism
chemical_list: D007770:L-Lactate Dehydrogenase; D003605:D-Amino-Acid Oxidase; D000637:Transaminases; C020649:glutamate-glyoxylate aminotransferase; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; C019635:Alanine-glyoxylate transaminase; C022638:serine-pyruvate aminotransferase