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[通过酶印记法纠正W/SSM品系大鼠遗传性半乳糖血症症状]

[Correction of the symptoms of hereditary galactosemia in W/SSM strain rats by enzymatic imprinting].

作者信息

Salganik R I, Solov'eva N A, Kandaurov V V

出版信息

Genetika. 1982 Mar;18(3):428-33.

PMID:7200439
Abstract

It was recently shown in this laboratory that treatment of newborn animals with certain enzymic inducers causes stable changes in the activities of the inducible enzymes at a later adult stage. Cataracts, hepato-splenomegaly and other galactosemia symptoms in galactosemic W/SSM rats develop spontaneously. The increased uptake of galactose by erythrocytes, but not the decreased level of galactose-1-phosphate uridyl transferase (Gal-1-PUT) activity was assumed to be the major cause of the disease. The administration of galactose to the newborn W/SSM rats (2 mg/g of body weight for 14 days) resulted in a sustained decline in the uptake of 14C-galactose by erythrocytes at least for five months, in an increase of glucoso-6-phosphate dehydrogenase activity and in a continuous fall of Gal-1-PUT activity. The neonatal treatment of the galactosemic rats with galactose abolished the main symptoms of galactosemia (cararacts, hepato-splenomegally) in adult animals, perhaps ar a consequence of the stable changes in the galactose metabolism.

摘要

最近本实验室研究表明,用某些酶诱导剂处理新生动物会导致成年后期诱导酶活性发生稳定变化。半乳糖血症的W/SSM大鼠会自发出现白内障、肝脾肿大及其他半乳糖血症症状。红细胞对半乳糖摄取增加,但1-磷酸半乳糖尿苷转移酶(Gal-1-PUT)活性降低并非该病的主要病因。给新生W/SSM大鼠注射半乳糖(2mg/g体重,持续14天),至少五个月内红细胞对14C-半乳糖的摄取持续下降,葡萄糖-6-磷酸脱氢酶活性增加,Gal-1-PUT活性持续下降。用半乳糖对患半乳糖血症的新生大鼠进行处理,消除了成年动物半乳糖血症的主要症状(白内障、肝脾肿大),这可能是半乳糖代谢稳定变化的结果。

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1
[Correction of the symptoms of hereditary galactosemia in W/SSM strain rats by enzymatic imprinting].[通过酶印记法纠正W/SSM品系大鼠遗传性半乳糖血症症状]
Genetika. 1982 Mar;18(3):428-33.
2
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