INORGANIC PYROPHOSPHATE IN PLASMA IN NORMAL PERSONS AND IN PATIENTS WITH HYPOPHOSPHATASIA, OSTEOGENESIS IMPERFECTA, AND OTHER DISORDERS OF BONE
INORGANIC PYROPHOSPHATE IN PLASMA IN NORMAL PERSONS AND IN PATIENTS WITH HYPOPHOSPHATASIA, OSTEOGENESIS IMPERFECTA, AND OTHER DISORDERS OF BONE
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DOI：
10.1172/jci106589
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发表时间：
1971-01-01
影响因子：
15.9
通讯作者：
FLEISCH, H
中科院分区：
文献类型：
--
作者：
RUSSELL, RGG;BISAZ, S;FLEISCH, H
本文报道了用~（32）P标记焦磷酸盐的同位素稀释法测定人血浆中无机焦磷酸盐（PP_1）的方法。通过离子交换色谱、纸色谱和无机焦磷酸酶孵育期间的洗脱模式评估，该方法的专属性优于90%。血浆PP 1单次估计值的99%置信限为± 13%。男性和女性之间的血浆PP 1没有差异，但年轻人（0-15岁）的值略高于老年人。73名男性和女性血浆中PP 1的平均浓度（±SE）为3.50 ±0.11 μ mol/L（0.217 ±0.007 μg P/ml）和正常范围（99%限度）为1.19-5.65 μ mol/L（0.074-0.350 μg P/ml）PP 1在体内外均能阻止磷酸钙的沉淀，并且可以减缓羟基磷灰石晶体生长和溶解的速率。因此，在几种骨疾病中测定了血浆PP 1。正常值见于骨生成障碍、骨硬化症、“急性”骨质疏松症和原发性甲状旁腺功能亢进。低磷酸酶血症患者血浆PP 1水平升高。血浆中PP 1的过量可能是低磷酸酶症矿化缺陷的原因，骨中碱性磷酸酶的功能可能是作为焦磷酸酶在钙沉积部位起作用。
An isotope dilution method, using32P-labeled pyrophosphate, has been developed for the measurement of inorganic pyrophosphate (PP1) in human plasma. The specificity of the method was better than 90% as assessed by elution patterns during ion-exchange chromatography, by paper chromatography, and by incubation with inorganic pyrophosphatase. The 99% confidence limits for a single estimation of plasma PP1was ±13%. There were no differences in plasma PP1between men and women, but the values in young people (0-15 yr) were slightly higher than in older people. The mean concentration (±SE) of PP1in the plasma of 73 men and women was 3.50 ±0.11 μmoles/liter (0.217 ±0.007 μg P/ml) and the normal range (99% limits) was 1.19-5.65 μmoles/liter (0.074-0.350 μg P/ml).It has been suggested that PP1may be important in calcium metabolism because PP1can prevent the precipitation of calcium phosphates in vitro and in vivo, and can slow the rates at which hydroxyapatite crystals grow and dissolve. Plasma PP1was therefore measured in several disorders of bone. Normal values were found in osteogenesis imperfecta, osteopetrosis, “acute” osteoporosis, and primary hyperparathyroidism. Plasma PP1was invariably raised in hypophosphatasia. The excess of PP1in plasma might be the cause of the defective mineralization in hypophosphatasia and the function of alkaline phosphatase in bone may be to act as a pyrophosphatase at sites of calcium deposition.