Foss I, Trygstad O
Acta Endocrinol (Copenh). 1975 Oct;80(2):398-416. doi: 10.1530/acta.0.0800398.
Urine from 5 patients with congenital generalized lipodystrophy has been fractionated by protein precipitation and Sephadex gel filtration. A fraction with a molecular weight in the range of 1000 was observed to be metabolically active in mice, rats, and rabbits. Hypophysectomized rats got hypoglycaemia following an injection, and the lipolytic-hyperglycaemic effect of ACTH was reduced after injection into intact mice. This effect was probably due to insulin release, because no insulin-like activity was observed on isolated fat cells in vitro. Persistant changes were observed in the animals after 3 weeks of daily injections of the urinary fraction. Adult mice and rabbits developed lipoatrophy with decrease of body weight in spite of a doubling of the food consumption. The metabolic rate and the body temperature were raised. Infantile animals developed a lipodystrophic state with increased growth velocity, and 50 per cent increase of the body weight, although no fat depots were observed. The treated animals got hyperglycaemia, hypertriglyceridaemia, hyperinsulinaemia, and insulin resistance. The rabbits developed manifest diabetes. The corresponding fraction prepared from the urine from the lipoatrophic rabbits produced lipoatrophy after injection into the mice. It is suggested that the lipodystrophic urinary fraction is of hypothalamic origin, and that it acts through the pituitary gland. The fraction is still heterogenous, and was observed to contain thyrotrophin releasing activity.
对5例先天性全身性脂肪营养不良患者的尿液进行了蛋白沉淀和葡聚糖凝胶过滤分级分离。观察到一种分子量在1000左右的组分在小鼠、大鼠和兔子体内具有代谢活性。切除垂体的大鼠注射后出现低血糖，将该组分注射到正常小鼠体内后，促肾上腺皮质激素的脂解 - 高血糖作用减弱。这种作用可能是由于胰岛素释放，因为在体外分离的脂肪细胞上未观察到胰岛素样活性。在每天注射该尿液组分3周后，动物出现持续变化。成年小鼠和兔子尽管食物摄入量增加了一倍，但仍出现脂肪萎缩和体重减轻。代谢率和体温升高。幼年动物出现脂肪营养不良状态，生长速度加快，体重增加50%，尽管未观察到脂肪储存。接受治疗的动物出现高血糖、高甘油三酯血症、高胰岛素血症和胰岛素抵抗。兔子出现明显的糖尿病。从脂肪萎缩兔子尿液中制备的相应组分注射到小鼠体内后也产生了脂肪萎缩。提示脂肪营养不良性尿液组分起源于下丘脑，且通过垂体发挥作用。该组分仍然是异质性的，并且观察到含有促甲状腺激素释放活性。
