Primary hyperoxaluria type 1 in Japan |
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Authors: | Arata Ichiyama Toshiaki Oda Eiko Maeda-Nakai |
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Institution: | (1) First Department of Biochemistry, Hamamatsu University School of Medicine, 3600 Handa-cho, Hamamatsu, 431-3192 Shizuoka, Japan |
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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 least in part, to the food
habit of Japanese, especially that of old generation, that they prefer boiled greens rather than frying or raw vegetables. |
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Keywords: | Primary hyperoxaluria type 1 oxalate glyoxylate glycolate liver peroxisomes serine:pyruvate/alanine:glyoxylate aminotransferase (alanine:glyoxylate aminotransferase) |
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