SIMULTANEOUS ABSENCE OF ALPHA-1,4-GLUCOSIDASE AND ALPHA-1,6-GLUCOSIDASE ACTIVITIES (PH 4) IN TISSUES OF CHILDREN WITH TYPE 2 GLYCOGEN STORAGE DISEASE
SIMULTANEOUS ABSENCE OF ALPHA-1,4-GLUCOSIDASE AND ALPHA-1,6-GLUCOSIDASE ACTIVITIES (PH 4) IN TISSUES OF CHILDREN WITH TYPE 2 GLYCOGEN STORAGE DISEASE
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DOI：
10.1021/bi00808a017
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发表时间：
1970-01-01
期刊：
影响因子：
2.9
通讯作者：
JEFFREY, PL
中科院分区：
文献类型：
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作者：
BROWN, BI;BROWN, DH;JEFFREY, PL
材料与方法在美国各地许多医生的慷慨合作下，通过活组织检查或在死亡后6小时内进行尸检从患者身上获得人体组织样本。小心确保组织立即冷冻，方法是浸入液氮中或将其压在干冰块之间。然后将它们放置在密闭的容器中以防止脱水，并将其包装在干冰中并运往圣刘易斯。到达后，将组织样本转移到小塑料袋中，将其在-90 ℃下冷冻保存在气密小瓶中，直至用于分析。在不同时间进行的重复测定的结果表明，当如上所述取出并储存人体组织时，糖原代谢的许多酶的活性良好地维持了6个月或更长的时间。然而，在实验室收到每个组织样本后，总是尽可能迅速地对其进行分析。如Jeffrey et al.（1970年a）。D-（+）-海藻糖和甲基-D-吡喃葡萄糖苷是Sigma Chemical Co.
Materials and MethodsMaterials. With thegenerous cooperation of numerous physicians throughout the United States human tissue samples were obtained from patients by biopsy or at autopsy per-formed within 6 hr after death. Care was taken to ensure that the tissues were frozen immediately, either by immersion in liquid nitrogen or by pressing them between blocks of Dry Ice. They were then placed in closed containers to prevent dehydration, and these were packed in Dry Ice and shipped to Saint Louis. Upon arrival, the tissue specimens were transferred to small, plastic bags which were kept frozen in air-tight vials at—90 until used for analysis. The results of repeated assays done at various times have shown that the activities of many enzymes of glycogen metabolism are well maintained for periods of 6 months or longer when human tissues are taken and stored as described above. How-ever, an effort was always made to analyze each tissue sample as promptly as possible after its receipt in the laboratory. The substrates, maltose, isomaltose, and glycogen were obtained and purified as described by Jeffrey et al.(1970a). D-(+)-Trehalose and methyl-D-glucopyranoside were prod-ucts of Sigma Chemical Co.