摘要
本文简单介绍了铜锌超氧化物歧化酶的结构、功能及其相互关系 ,较为详细地讨论了与神经退行性紊乱 ,尤其是与肌萎性脊髓侧索硬化症有关的铜锌超氧化物歧化酶突变体的生物无机化学的研究进展 ,提出由该酶突变而引起的蛋白质局部结构变化和金属离子缺失 ,以及由此导致的氧化损伤和聚集作用可能是导致神经退行性紊乱的主要原因之一。
The structure function relationships of CuZn superoxide dismutases (SOD1) are briefly reviewed. The biological inorganic chemistry of SOD1 mutants and neurodegenerative diseases, in particular the amyotrophic lateral sclerosis (ALS) is discussed in detail. It's reasonable to propose that the oxidative damage and aggregation resulted from the structural and metal binding variations in SOD1 mutants are one of the key factors that cause ALS.
出处
《化学进展》
SCIE
CAS
CSCD
2004年第5期813-819,共7页
Progress in Chemistry
