Hepatic glucokinase regulatory protein and carbohydrate response element binding protein attenuation reduce de novo lipogenesis but do not mitigate intrahepatic triglyceride accumulation in Aldob deficiency

Autor: Amée M. Buziau, Maaike H. Oosterveer, Kristiaan Wouters, Trijnie Bos, Dean R. Tolan, Loranne Agius, Brian E. Ford, David Cassiman, Coen D.A. Stehouwer, Casper G. Schalkwijk, Martijn C.G.J. Brouwers

Publikováno v: Molecular Metabolism, Vol 87, Iss , Pp 101984- (2024)

Objective: Stable isotope studies have shown that hepatic de novo lipogenesis (DNL) plays an important role in the pathogenesis of intrahepatic lipid (IHL) deposition. Furthermore, previous research has demonstrated that fructose 1-phosphate (F1P) no

Externí odkaz: https://doaj.org/article/f788e7cbbd6b437a8180795a7483cdf6

Insights into pathogenesis of five novel GCK mutations identified in Chinese MODY patients

Autor: Ming Li, Chen Chen, Weijing Zhao, Langen Zhuang, Xiaoxu Ge, Yanyan Jiang, Yanjun Liu, Rong Zhang, Taishan Zheng, Xipeng Liu, Yanzhong Wang, Juan Zhang, Jun Yin, Yating Chen, Meisheng Jiang, Di Yang, Ming Lu, Limei Liu

Publikováno v: Liu, L, Liu, Y, Ge, X, Liu, X, Chen, C, Wang, Y, Li, M, Yin, J, Zhang, J, Chen, Y, Zhang, R, Jiang, Y, Zhao, W, Yang, D, Zheng, T, Lu, M, Zhuang, L & Jiang, M 2018, ' Insights into pathogenesis of five novel GCK mutations identified in Chinese MODY patients ', Metabolism: clinical and experimental, vol. 89, pp. 8-17 . https://doi.org/10.1016/j.metabol.2018.09.004

Objective Heterozygous inactivating mutations in GCK are associated with defects in pancreatic insulin secretion and/or hepatic glycogen synthesis leading to mild chronic hyperglycemia of maturity onset diabetes of young type 2 (MODY2). However, the

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3c466ba683efa67286ea719df4658a78
https://doi.org/10.1016/j.metabol.2018.09.004

Kidney and vascular function in adult patients with hereditary fructose intolerance

Autor: Carla E. M. Hollak, Edith J. M. Feskens, Casper G. Schalkwijk, David Cassiman, François-Guillaume Debray, Jörgen Bierau, Nynke Simons, Alfons J.H.M. Houben, Judith A P Bons, Coen D.A. Stehouwer, Martijn C. G. J. Brouwers, Nicolaas C. Schaper

Publikováno v: Molecular Genetics and Metabolism Reports, 23:100600. Elsevier Science
Molecular Genetics and Metabolism Reports, 23
Molecular Genetics and Metabolism Reports
Molecular Genetics and Metabolism Reports 23 (2020)
Molecular Genetics and Metabolism Reports, Vol 23, Iss, Pp-(2020)
Molecular genetics and metabolism reports, 23:100600. Elsevier BV

Objective: Previous studies have shown that patients with hereditary fructose intolerance (HFI) are characterized by a greater intrahepatic triglyceride content, despite a fructose-restricted diet. The present study aimed to examine the long-term con

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4cf02529936cb64373d65e3edaf82789
https://cris.maastrichtuniversity.nl/en/publications/147d680d-22ab-48b1-84b7-18f928107a37

Analysis of the co-operative interaction between the allosterically regulated proteins GK and GKRP using tryptophan fluorescence

Autor: Zelent, B, Raimondo, A, Barrett, A, Buettger, CW, Chen, P, Gloyn, AL, Matschinsky, FM

Publikováno v: Biochemical Journal

Hepatic glucose phosphorylation by GK (glucokinase) is regulated by GKRP (GK regulatory protein). GKRP forms a cytosolic complex with GK followed by nuclear import and storage, leading to inhibition of GK activity. This process is initiated by low gl

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::8c52f5bf25955caf481b7facf359cbf8
https://doi.org/10.1042/bj20131363

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