Sep 9, 2023 · Fumarate hydratase (FH) is an enzyme of the Tricarboxylic Acid (TCA) cycle whose mutations lead to hereditary and sporadic forms of cancer.

延胡索酸水化酶 （FH）是参与三羧酸（TCA）循环的代谢酶，催化延胡索酸盐向苹果酸盐的可逆转化，已被证实是一种肿瘤抑制因子，FH的突变可导致 遗传性平滑肌瘤病肾癌 （HLRCC，一种以侵袭性肾癌为特征的综合征）。 FH缺陷细胞和肿瘤的一个特点是延胡索酸盐的异常积累，其可通过一系列致癌级联的激活来驱动恶性转化和肿瘤进展 【1,2】。 然而，尽管目前已经清楚FH缺失的长期后果，但是其急性反应尚未可知；而且当人们在肿瘤或其他细胞模型中分析时，所有那些 …

Mar 8, 2023 · Fumarate hydratase (FH), a metabolic enzyme of the tricarboxylic acid (TCA) cycle that catalyses the reversible conversion of fumarate to malate, has been identified as a...

May 25, 2023 · 富马酸水合酶（fh）为三羧酸（tca）循环的一种代谢酶，已被确定为真正的肿瘤抑制因子。 FH的缺失会导致富马酸的积累，进而激活一系列致癌级联反应驱动恶性转化和肿瘤进展，如遗传性平滑肌瘤病和肾细胞癌综合征相关性肾细胞癌（HLRCC）。

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 ...

Jun 1, 2024 · Fumarate hydratase (FH) and fumarate are now known to regulate cytokine production. Recent findings suggest FH loss leads to mitochondrial nucleic acid release, leading to type I interferon production. Tricarboxylic acid (TCA) cycle metabolites have been implicated in modulating signalling pathways in immune cells.

Fumarate hydratase (FH) is an evolutionary conserved TCA cycle enzyme that reversibly catalyzes the hydration of fumarate to L-malate and has a moonlight function in the DNA damage response (DDR). Interestingly, FH has a contradictory cellular function, as it is pro-survival through its role in the TCA cycle, yet its loss can drive tumorigenesis.

Aug 3, 2020 · Fumarate hydratase (FH) is an enzyme in the tricarboxylic acid (TCA) cycle, biallelic loss-of-function mutations of which are associated with hereditary leiomyomatosis...

同样，通过使用 fh-in-1（一种 fh 抑制剂 (fhi)）积累内在富马酸盐，以浓度和时间依赖性方式强烈抑制 cd8+ t 细胞活化。 进一步分析表明，这种内源性和外源性富马酸盐积累的作用只发生在刺激期间，而不是刺激后，这表明富马酸盐调节幼稚 CD8+ T 细胞向活性状态 ...

Several cancers harbor mutations that affect the integrity of this cycle, mostly at the levels of isocitrate dehydrogenase (IDH), succinate dehydrogenase (SDH), and fumarate hydratase (FH). This results in dysregulation in the production of TCA cycle metabolites and is probably implicated in cancer initiation.