中文摘要：

线粒体琥珀酸脱氢酶（succinate dehydrogenase, SDH）是三羧酸循环和有氧电子传递呼吸链中的关键酶之一，包含A、B、C、D4个亚基．4个亚基分别由4个基因编码，即SDHA、SDHB、SDHC和SDHD，4个基因突变可以诱发癌症，包括副神经节瘤（paragan glioma，PGL）、嗜铬细胞瘤（pheochromocytoma，PHEO）、肾细胞癌（renal cell carcinoma, RCC）、胃肠道间质瘤（gastrointestinal stromal tumors, GIST）、Leigh综合症等．近年来，突变的SDH已经被证实是一种重要的诊断与预后的生物标志物和治疗分子标靶．本文就SDH存在的各种突变以及在肿瘤发生、发展与转移的作用机理研究的进展进行全面的论述．

英文摘要：

Succinate dehydrogenase （SDH）, a cruciral multiprotein enzymatic complex in the Krebs cycle/transport chain, is located in the cristate of the mitochondria, and composed of A, B, C, and D subunits, which are encoded by SDHA, SDHB, SDHC, and SDHD, respectively. The SDH mutations play a crucial oncogenic role in paraganglioma （PGL, types 1-5）, pheochromocytoma （PHEO）, renal cell carcinoma （RCC）, gastrointestinal stromal manors （GIST）, rare hypophyseal adenomas, and Leigh syndromes. Mutated SDH has been proved to be an important biomarker for diagnosis and a therapeutic target in these cancers. This review summarized and updated a variety of SDH mutations in the neoplasms and discussed the oncogenic role of SDH mutants in proliferation, apoptosis, invasion, migration, tumorgenesis and senescence.