Structural basis of fumarate hydratase deficiency.

Structural basis of fumarate hydratase deficiency.

Show other versions (2)

Fumarate hydratase catalyzes the stereospecific hydration across the olefinic double bond in fumarate leading to L-malate. The enzyme is expressed in mitochondrial and cytosolic compartments, and participates in the Krebs cycle in mitochondria, as well as in regulation of cytosolic fumarate levels....

Fuld beskrivelse

Bibliografiske detaljer
Main Authors:	Picaud, S, Kavanagh, K, Yue, W, Lee, W, Muller-Knapp, S, Gileadi, O, Sacchettini, J, Oppermann, U
Format:	Journal article
Sprog:	English
Udgivet:	2011

Lignende værker

Structural basis of fumarate hydratase deficiency.
af: Picaud, S, et al.
Udgivet: (2011)

Structural basis of fumarate hydratase deficiency.
af: Picaud, S, et al.
Udgivet: (2011)

Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency
af: Ternette, N, et al.
Udgivet: (2013)

Inhibition of mitochondrial aconitase by succination in fumarate hydratase deficiency.
af: Ternette, N, et al.
Udgivet: (2013)

Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency
af: Nicola Ternette, et al.
Udgivet: (2013-03-01)

Fumarate hydratase deficiency and cancer: activation of hypoxia signaling?
af: Ratcliffe, P
Udgivet: (2007)

Case report: Uterine leiomyoma with fumarate hydratase deficiency
af: Diana Bužinskienė, et al.
Udgivet: (2024-05-01)

Research progress on fumarate hydratase-deficient unterine leiomyoma
af: Wang Qing, Zheng Jingying, Ding Lianying, Zhao Weixin, Wang Mengqi, Zhao Shuhua
Udgivet: (2022-01-01)

Distinct expression profile in fumarate-hydratase-deficient uterine fibroids.
af: Vanharanta, S, et al.
Udgivet: (2006)

Radiologic features of fumarate hydratase-deficient renal cell carcinoma
af: XIE Qiao, et al.
Udgivet: (2023-10-01)

Targeting strategies in the treatment of fumarate hydratase deficient renal cell carcinoma
af: Andrea Katharina Lindner, et al.
Udgivet: (2022-07-01)

How a woman’s myomectomy saved her father’s life: evidence of fumarate hydratase–deficient uterine leiomyoma and early detection of germline variants in fumarate hydratase
af: Greysha Rivera-Cruz, M.D., et al.
Udgivet: (2022-03-01)

Dysregulation of hypoxia pathways in fumarate hydratase-deficient cells is independent of defective mitochondrial metabolism.
af: O'Flaherty, L, et al.
Udgivet: (2010)

Fumarate hydratase-deficient renal-cell carcinoma: Report of a rare case
af: Pan Niu, et al.
Udgivet: (2025-01-01)

Expression profiling in progressive stages of fumarate-hydratase deficiency: the contribution of metabolic changes to tumorigenesis.
af: Ashrafian, H, et al.
Udgivet: (2010)

High-intensity focused ultrasound ablation in the treatment of fumarate hydratase-deficient uterine leiomyoma
af: Luyao Zhang, et al.
Udgivet: (2024-12-01)

Fumarate hydratase-deficient renal cell carcinoma complicated with liver metastasis: case report
af: Hanmin Chen, et al.
Udgivet: (2024-11-01)

Fumarate hydratase-deficient renal cell carcinoma: a case report and review of the literature
af: Yanting Lv, et al.
Udgivet: (2023-04-01)

Crystal structure of an Fe-S cluster-containing fumarate hydratase enzyme from
af: Nonato, M. Cristina, et al.
Udgivet: (2017)

Haem oxygenase is synthetically lethal with the tumour suppressor fumarate hydratase.
af: Frezza, C, et al.
Udgivet: (2011)

Haem oxygenase is synthetically lethal with the tumour suppressor fumarate hydratase
af: Frezza, C, et al.
Udgivet: (2011)

Case report: A rare low-grade fumarate hydratase-deficient renal cell carcinoma
af: Di Sun, et al.
Udgivet: (2024-11-01)

Aberrant succination of proteins in fumarate hydratase-deficient mice and HLRCC patients is a robust biomarker of mutation status.
af: Bardella, C, et al.
Udgivet: (2011)

Aberrant succination of proteins in fumarate hydratase-deficient mice and HLRCC patients is a robust biomarker of mutation status.
af: Bardella, C, et al.
Udgivet: (2011)

The TCA cycle and tumorigenesis: the examples of fumarate hydratase and succinate dehydrogenase.
af: Pollard, P, et al.
Udgivet: (2003)

Fumarate hydratase deletion in pancreatic beta cells leads to progressive diabetes
af: Adam, J, et al.
Udgivet: (2017)

Genomic and transcriptomic features between primary and paired metastatic fumarate hydratase–deficient renal cell carcinoma
af: Jiayu Liang, et al.
Udgivet: (2023-05-01)

Fumarate Hydratase Deletion in Pancreatic β Cells Leads to Progressive Diabetes
af: Julie Adam, et al.
Udgivet: (2017-09-01)

A role for cytosolic fumarate hydratase in urea cycle metabolism and renal neoplasia.
af: Adam, J, et al.
Udgivet: (2013)

Fumarate hydratase: a new checkpoint of metabolic regulation in inflammatory macrophages
af: Aicha Jeridi, et al.
Udgivet: (2023-09-01)

Synthetically Lethal Interactions of Heme Oxygenase-1 and Fumarate Hydratase Genes
af: Paulina Podkalicka, et al.
Udgivet: (2020-01-01)

The role(s) of hypoxia-inducible factors (HIFs) in the development of renal cysts in mice deficient in fumarate hydratase (Fh1)
af: Hatipoglu, E
Udgivet: (2011)

Circulating succinate-modifying metabolites accurately classify and reflect the status of fumarate hydratase–deficient renal cell carcinoma
af: Liang Zheng, et al.
Udgivet: (2023-06-01)

Fumarate hydratase-deficient renal cell carcinoma in kidney transplant patients: one case report and literature review
af: WANG Yueming, et al.
Udgivet: (2023-07-01)

HIF overexpression correlates with biallelic loss of fumarate hydratase in renal cancer: novel role of fumarate in regulation of HIF stability.
af: Isaacs, J, et al.
Udgivet: (2005)

A Role for Cytosolic Fumarate Hydratase in Urea Cycle Metabolism and Renal Neoplasia
af: Julie Adam, et al.
Udgivet: (2013-05-01)

Crystal Structures of Fumarate Hydratases from Leishmania major in a Complex with Inhibitor 2-Thiomalate
af: Feliciano, Patricia Rosa, et al.
Udgivet: (2020)

Bilateral fumarate hydratase deficient renal cell carcinoma in a patient with hereditary leiomyomatosis and renal cell cancer syndrome
af: Akihiro Ono, et al.
Udgivet: (2024-03-01)

Adult leydig cell tumors of the testis caused by germline fumarate hydratase mutations.
af: Carvajal-Carmona, L, et al.
Udgivet: (2006)

A Case of Reed Syndrome with a Novel Mutation in the Fumarate Hydratase Gene
af: Christin B. Laufer, et al.
Udgivet: (2013-01-01)