Chodirker B N, Coburn S P, Seargeant L E, Whyte M P, Greenberg C R
Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Canada.
J Inherit Metab Dis. 1990;13(6):891-6. doi: 10.1007/BF01800216.
We measured plasma levels of pyridoxal-5'-phosphate (PLP), a cofactor form of vitamin B6 and apparent natural substrate for alkaline phosphatase (ALP), in carriers and in non-carriers of the severe perinatal and infantile forms of hypophosphatasia, both before and after an oral load of pyridoxine (i.e. 1/3 mg/kg body weight). The assignment of carrier status was determined by serum ALP activity, level of serum inorganic phosphate, and if necessary urinary phosphoethanolamine excretion. Plasma PLP levels were significantly increased in the carriers both before and especially after B6 loading.
我们测定了重度围产期和婴儿型低磷酸酯酶症携带者和非携带者体内维生素B6的辅因子形式——磷酸吡哆醛（PLP）的血浆水平，它也是碱性磷酸酶（ALP）的天然底物，分别在口服吡哆醇（即1/3毫克/千克体重）之前和之后进行测定。携带者状态通过血清碱性磷酸酶活性、血清无机磷酸盐水平以及必要时的尿磷酸乙醇胺排泄来确定。无论是在服用B6之前，尤其是在服用B6之后，携带者的血浆PLP水平均显著升高。
