ABNORMAL METHIONINE ADENOSYLTRANSFERASE IN HYPERMETHIONINEMIA

ABNORMAL METHIONINE ADENOSYLTRANSFERASE IN HYPERMETHIONINEMIA
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DOI:
10.1016/0006-291x(75)90527-6
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发表时间:
1975-01-01
影响因子:
3.1
通讯作者:
MARTIN, JJ
MARTIN, JJ
中科院分区:
生物学4区
文献类型:
--
作者:
FINKELSTEIN, JD;KYLE, WE;MARTIN, JJ

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高蛋氨酸血症儿童肝脏蛋氨酸腺苷转移酶活性降低。动力学研究表明,来自大鼠和人肝脏的酶具有负协同作用。患者的酶表现为表观最大Km(met)下降。该突变酶的特点可以解释临床异常。
Methionine adenosyltransferase activity was reduced in liver from a child with hypermethioninemia. Kinetic studies demonstrated a pattern suggesting negative cooperativity for enzyme derived from rat and human liver. The patient's enzyme was characterized by a decrease in the apparent maximum Km(met). The characteristics of this mutant enzyme could explain the clinical abnormalities.