Schiaffino S, Hanzlíková V
J Cell Biol. 1972 Jan;52(1):41-51. doi: 10.1083/jcb.52.1.41.
Large amounts of glycogen accumulate in rat skeletal muscle fibers during the late fetal stages and are mobilized in the first postnatal days. This glycogen depletion is relatively slow in the immature leg muscles, in which extensive deposits are still found 24 hr after birth and, to some extent, persist until the 3rd day. In the more differentiated psoas muscle and especially in the diaphragm, the glycogen stores are completely mobilized already during the early hours. Section of the sciatic nerve 3 days before birth or within the first 2 hr after delivery does not affect glycogen depletion in the leg muscles. Neonatal glycogenolysis in rat muscle fibers takes place largely by segregation and digestion of glycogen particles in autophagic vacuoles. These vacuoles: (a) are not seen in fetal muscle fibers or at later postnatal stages, but appear concomitantly with the process of glycogen depletion and disappear shortly afterwards; (b) are prematurely formed in skeletal muscles of fetuses at term treated with glucagon; (c) contain almost exclusively glycogen particles and no other recognizable cell constituents; (d) have a double or, more often, single limiting membrane and originate apparently from flattened sacs sequestering glycogen masses; (e) are generally found to contain reaction product in preparations incubated from demonstration of acid phosphatase activity. The findings emphasize the role of the lysosomal system in the physiological process of postnatal glycogen mobilization and appear relevant in the interpretation of type II glycogen storage disease.
在胎儿后期,大量糖原积聚在大鼠骨骼肌纤维中,并在出生后的头几天被动员起来。这种糖原消耗在未成熟的腿部肌肉中相对较慢,在出生后24小时仍能发现大量糖原沉积,并且在一定程度上会持续到第3天。在分化程度更高的腰大肌,尤其是膈肌中,糖原储备在早期几个小时内就已被完全动员。在出生前3天或出生后2小时内切断坐骨神经,不会影响腿部肌肉中的糖原消耗。大鼠肌肉纤维中的新生儿糖原分解主要通过自噬泡中糖原颗粒的分离和消化来进行。这些自噬泡:(a) 在胎儿肌肉纤维或出生后的后期阶段看不到,但随着糖原消耗过程同时出现,并在不久后消失;(b) 在足月用胰高血糖素处理的胎儿骨骼肌中过早形成;(c) 几乎只含有糖原颗粒,没有其他可识别的细胞成分;(d) 有双层膜,更常见的是单层膜,显然起源于隔离糖原团块的扁平囊泡;(e) 在用于显示酸性磷酸酶活性的孵育制剂中,通常发现含有反应产物。这些发现强调了溶酶体系统在出生后糖原动员生理过程中的作用,并且似乎与II型糖原贮积病的解释相关。