星质和胞质5 ' -nucleotidases在正常和AMP deaminase-deficient人类骨骼肌。
文章的细节
-
引用
复制到剪贴板 -
Hanisch F, Hellsten Y, Zierz年代
星质和胞质5 ' -nucleotidases在正常和AMP deaminase-deficient人类骨骼肌。
生物化学杂志。2006年1月,387 (1):53-8。
- PubMed ID
-
16497164 (在PubMed]
- 文摘
-
在骨骼肌,腺苷酸(AMP)是主要由腺苷酸脱氨酶脱氨基。然而,C34T AMPD1基因的突变严重降低了腺苷酸脱氨酶的活动。另外,细胞内的安保是脱去磷酸腺苷通过胞质AMP 5核苷酸酶(cN-I)。与纯合个体C34T突变,cN-I可能更AMP去除的重要途径。cN-I我们确定活动的腺苷酸脱氨酶,胞质总5的核苷酸酶(cN)、ecto-5的核苷酸酶(户籍纪录)和全匀浆5核苷酸酶活动的骨骼肌活检患者不同AMPD1基因型(比如C34T突变(TT);C34T突变杂合子(CT);以及该为野生型(CC):病变控制CC;和正常对照组CC]。腺苷酸脱氨酶活性显示genotype-dependent差异。正常对照组总cN活动占整个匀浆的57 + / - -22% 5核苷酸酶活动,与其他组没有明显不同。 A weak inverse correlation was found between AMP deaminase and cN-I activities (r2=0.18, p<0.01). There were no significant differences between different groups in the activities of cN-I, whole homogenate 5'-nucleotidase and ectoN, or in cN-I expression on Western blots. No correlation for age, fibre type distribution and AMPD1 genotype was found for whole homogenate nucleotidase, total cN and cN-I using multiple linear regression analysis. There was no gender-specific difference in the activities of whole homogenate nucleotidase, total cN and cN-I. The results indicate no changes in the relative expression or catalytic behaviour of cN-I in AMP deaminase-deficient human skeletal muscle, but suggest that increased turnover of AMP by cN-I in working skeletal muscle is due to higher substrate availability of AMP.