Sohar I, Laszlo A, Gaal K, Mechler F
Department of Biochemistry, University Medical School, Szeged, Hungary.
Biol Chem Hoppe Seyler. 1988 May;369 Suppl:277-9.
Lysosomal cysteine proteinase (cathepsin B, H, and L) and MMP-7ase muscle metalloproteinase activities were measured in serum from Duchenne muscular dystrophic male patients and their mothers as gene-carriers. The activity of cathepsin H significantly increased in the Duchenne muscular dystrophic (DMD)-hemizygotes group and in the group of DMD heterozygotes. Significant positive correlation was found between the activity of serum creatine kinase (which previously has been proven to be a marker of muscular dystrophy) and of cathepsin L in the DMD-hemizygotes group. Furthermore, correlations were found between the activity of creatine kinase and MMP-7ase or between activity of creatine kinase and cathepsin H in the DMD heterozygotes. The changes in activity of proteolytic enzymes in serum of dystrophic patients can be explained by the elevated proteolytic enzyme activity in dystrophic muscle observed previously.
在杜兴氏肌营养不良男性患者及其作为基因携带者的母亲的血清中，检测了溶酶体半胱氨酸蛋白酶（组织蛋白酶B、H和L）以及肌肉金属蛋白酶MMP-7的活性。组织蛋白酶H的活性在杜兴氏肌营养不良（DMD）半合子组和DMD杂合子组中显著增加。在DMD半合子组中，血清肌酸激酶（此前已被证明是肌肉营养不良的标志物）的活性与组织蛋白酶L的活性之间存在显著正相关。此外，在DMD杂合子中，发现肌酸激酶的活性与MMP-7之间存在相关性，或者肌酸激酶的活性与组织蛋白酶H之间存在相关性。营养不良患者血清中蛋白水解酶活性的变化可以用先前观察到的营养不良肌肉中蛋白水解酶活性升高来解释。
