醇酸水合酶（FH）是三羧酸循环（TCA）中的一种酶，其突变导致遗传性和散发性癌症。自20多年以来，FH被发现是遗传性平滑肌瘤与肾细胞癌（HLRCC）癌症综合症的主要原因，但尚不清楚FH的丧失如何以组织特异性和具有攻击性行为的方式导致癌症。已经证明，通过醇酸基质醇酸的积累，FH的丧失可以激活一系列致癌级联反应，这对于转化的贡献仍在调查中。在本综述中，我们将综合总结这些最新发现，并提出了理解FH的生物学以及其突变如何促进转化将对建立新的癌代谢范式至关重要的观点。© 2023. 作者。

Fumarate hydratase (FH) is an enzyme of the Tricarboxylic Acid (TCA) cycle whose mutations lead to hereditary and sporadic forms of cancer. Although more than twenty years have passed since its discovery as the leading cause of the cancer syndrome Hereditary leiomyomatosis and Renal Cell Carcinoma (HLRCC), it is still unclear how the loss of FH causes cancer in a tissue-specific manner and with such aggressive behaviour. It has been shown that FH loss, via the accumulation of FH substrate fumarate, activates a series of oncogenic cascades whose contribution to transformation is still under investigation. In this review, we will summarise these recent findings in an integrated fashion and put forward the case that understanding the biology of FH and how its mutations promote transformation will be vital to establish novel paradigms of oncometabolism.© 2023. The Author(s).