Anaplerotic molecules: Current and future

Anaplerotic molecules: Current and future
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DOI:
10.1007/s10545-006-0320-1
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发表时间:
2006-04-01
影响因子:
4.2
通讯作者:
Roe, Charles R.
Roe, Charles R.
中科院分区:
医学2区
文献类型:
--
作者:
Brunengraber, Henri;Roe, Charles R.

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本文介绍了与柠檬酸循环运行调节相关的回补和分解的概念。回补是循环中携带乙酰辅酶A的催化中间体在氧化时的再填充。主要的回补底物是丙酮酸、谷氨酰胺/谷氨酸和丙酰辅酶A的前体(奇数链脂肪酸、特定氨基酸、C-5-酮体)。通过从柠檬酸循环中去除多余的中间产物,退化作用平衡了回补作用。主要回补底物的性质进行了审查,从潜在的临床应用的角度来治疗一些遗传性和后天性的条件。
This review presents the concepts of anaplerosis and cataplerosis in relation to the regulation of citric acid cycle operation. Anaplerosis is the re-filling of the catalytic intermediates of the cycle that carry acetyl-CoA as it is oxidized. The main anaplerotic substrates are pyruvate, glutamine/glutamate and precursors of propionyl-CoA (odd-chain fatty acids, specific amino acids, C-5-ketone bodies). Cataplerosis balances anaplerosis by removing excess intermediates from the citric acid cycle. The properties of the main anaplerotic substrates are reviewed from the point of view of potential clinical applications to the treatment of some inherited and acquired conditions.