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与家族性癫痫相关的低触珠蛋白血症

Hypohaptoglobinemia associated with familial epilepsy.

作者信息

Panter S S, Sadrzadeh S M, Hallaway P E, Haines J L, Anderson V E, Eaton J W

出版信息

J Exp Med. 1985 Apr 1;161(4):748-54. doi: 10.1084/jem.161.4.748.

Abstract

In select kindreds afflicted with familial idiopathic epilepsy, most individuals suffering seizures also have low levels of the plasma hemoglobin-binding protein, haptoglobin. This hypohaptoglobinemia may be causally associated with a tendency to develop epilepsy. Our experimental results indicate that artificially-induced hypohaptoglobinemia in mice causes retarded clearance of free hemoglobin from the central nervous system, and that such free hemoglobin may engender the peroxidation of brain lipids. We hypothesize that hypohaptoglobinemia, either inherited, or acquired via traumatic processes, may prevent efficient clearance of interstitial hemoglobin from the central nervous system, thereby predisposing these people to encephalic inflammation and the appearance of seizure disorders.

摘要

在一些患有家族性特发性癫痫的家族中,大多数癫痫发作的个体血浆血红蛋白结合蛋白——触珠蛋白水平也较低。这种低触珠蛋白血症可能与癫痫发病倾向存在因果关系。我们的实验结果表明,人工诱导小鼠出现低触珠蛋白血症会导致中枢神经系统中游离血红蛋白的清除延迟,并且这种游离血红蛋白可能会引发脑脂质的过氧化。我们推测,无论是遗传性的还是通过创伤过程获得的低触珠蛋白血症,都可能阻碍中枢神经系统间质血红蛋白的有效清除,从而使这些人易患脑部炎症和癫痫发作紊乱。

相似文献

3
[Epilepsy and hypohaptoglobinemia].[癫痫与低触珠蛋白血症]
Zhonghua Shen Jing Jing Shen Ke Za Zhi. 1992 Apr;25(2):88-91, 126.

引用本文的文献

本文引用的文献

1
Haptoglobin types in British, Spanish Basque and Nigerian African populations.
Nature. 1958 Mar 22;181(4612):824-5. doi: 10.1038/181824a0.
3
A novel and specific method for the purification of hemoglobin-binding proteins.
Anal Biochem. 1981 Dec;118(2):353-7. doi: 10.1016/0003-2697(81)90593-5.
4
Sex limited ahaptoglobinaemia.性连锁无结合珠蛋白血症
Hum Genet. 1981;58(3):294-7. doi: 10.1007/BF00294926.

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