Silverstein E, Friedland J
Clin Chim Acta. 1977 Jan 3;74(1):21-5. doi: 10.1016/0009-8981(77)90382-5.
In adult chronic non-neuronopathic (Type 1) Gaucher's disease significant (p less than 0.001) elevations of angiotensin converting enzyme in serum (93.3 +/- 14.8 nmol/min/ml; number elevated, 8/11; normal control 32.2 +/- 1.30, n = 58) and spleen (5.62 +/- 0.35 nmol/min/mg protein, n = 2; control, 0.431 +/- 0.101, n = 4) and serum lysozyme (15.6 +/- 3.37 mug/ml; number elevated, 4/5) were observed. The KM for hippuryl-L-histidyl-L-leucine of Gaucher (1.31 mM) and normal (1.23 mM) serum angiotensin converting enzyme were similar. The increased angiotensin converting enzyme (ACE) in Gaucher's disease may be related to the genetic defect resulting in increased ACE synthesis in Gaucher cells, or perhaps generally, while high lysozyme may reflect an increased body mass of reticuloendothelial cells. These enzyme elevations may be of use in suggesting the possible presence of Gaucher's disease and perhaps in assessing the magnitude of pathologic involvement.
在成年慢性非神经元病变型（1型）戈谢病患者中，观察到血清中血管紧张素转换酶显著升高（p<0.001）（93.3±14.8nmol/（min·ml）；升高例数为8/11；正常对照为32.2±1.30，n=58），脾脏中血管紧张素转换酶也显著升高（5.62±0.35nmol/（min·mg蛋白），n=2；对照为0.431±0.101，n=4），血清溶菌酶也升高（15.6±3.37μg/ml；升高例数为4/5）。戈谢病患者血清血管紧张素转换酶对马尿酰-L-组氨酰-L-亮氨酸的米氏常数（1.31mM）与正常血清血管紧张素转换酶的米氏常数（1.23mM）相似。戈谢病中血管紧张素转换酶（ACE）升高可能与导致戈谢细胞中ACE合成增加的基因缺陷有关，或者可能是普遍现象，而高溶菌酶可能反映了网状内皮细胞总体质量增加。这些酶的升高可能有助于提示戈谢病的可能存在，或许还可用于评估病理累及程度。
