Primary hyperoxaluria type 1 in Japan

Cell Biochemistry and Biophysics
A IchiyamaE Maeda-Nakai

Abstract

Glyoxylate is an immediate precursor of oxalate, but in its metabolism the conversion into glycine catalyzed by serine:pyruvate/alanine:glyoxylate aminotransferase (SPT/AGT) appears to be the main route. When SPT/AGT is missing as in the case of primary hyperoxaluria type 1 (PH1) more glyoxylate is used for the oxalate production, resulting in calcium oxalate urolithiasis and finally systemic oxalosis. SPT/AGT is a unique enzyme of species-specific dual organelle localization; it is located largely in mitochondria in carnivores and entirely in peroxisomes in herbivores and man. For herbivores, the peroxisomal localization of SPT/AGT is indispensable to avoid massive production of oxalate, probably because liver peroxisomes are the main site of glyoxylate production from glycolate, and plants contain glycolate much more than animal tissues. Recently, we took charge of laboratory examination for 8 cases of primary hyperoxaluria in Japan, and felt that symptoms of some of the Japanese PH1 patients are apparently milder than those of Western patients. The reason of this is not clear, but from the above mentioned seemingly indispensable association of grass-eating with the peroxisomal localization of SPT/AGT it may be related, at le...Continue Reading

Citations

Jan 15, 2011·Journal of Nutrition and Metabolism·Chantal BémeurRoger F Butterworth
Jun 18, 2005·American Journal of Nephrology·Tatsuya TakayamaSeiichiro Ozono
May 16, 2002·Current Rheumatology Reports·Irama MaldonadoAntonio J Reginato

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