Results 31 to 40 of about 13,285 (168)

Mevalonate kinase deficiency

Neurology, 2004
Both mevalonic aciduria, characterized by psychomotor retardation, cerebellar ataxia, recurrent fever attacks, and death in early childhood, and hyper-immunoglobulin D (hyper-IgD) syndrome, with recurrent fever attacks without neurologic symptoms, are caused by a functional deficiency of mevalonate kinase.
Simon, A., Kremer, H.P.H., Wevers, R.A., Scheffer, H., Jong, J.G.N. de, Meer, J.W.M. van der, Drenth, J.P.H.   +6 more
openaire   +4 more sources

Mevalonate kinase is a cytosolic enzyme in humans [PDF]

Journal of Cell Science, 2004
In the past decade several reports have appeared which suggest that peroxisomes play a central role in isoprenoid/cholesterol biosynthesis. These suggestions were based primarily on the reported finding of several of the enzymes of the presqualene segment of the biosynthetic pathway in peroxisomes.
Hogenboom, Sietske, Tuyp, John J. M., Espeel, Marc, Koster, Janet, Wanders, Ronald J. A., Waterham, Hans R.   +5 more
openaire   +2 more sources

Characterization of phosphomevalonate kinase: chromosomal localization, regulation, and subcellular targeting

Journal of Lipid Research, 1999
Phosphomevalonate kinase catalyzes the conversion of mevalonate-5-phosphate to mevalonate-5-diphosphate and was originally believed to be a cytosolic enzyme. In this study we have localized the phosphomevalonate kinase gene to chromosome 1p13–1q22–23 and
Lisa M. Olivier, Ken L. Chambliss, K. Michael Gibson, Skaidrite K. Krisans   +3 more
doaj   +1 more source

Determination of mevalonate in blood plasma in man and rat. Mevalonate “tolerance” tests in man.

Journal of Lipid Research, 1979
A method is described for the determination of mevalonate in ultrafiltrates of blood plasma. The method depends on the phosphorylation of mevalonate with [gamma-32P]ATP and mevalonate kinase to 5-[32P]phosphomevalonate, and the subsequent isolation of ...
G Popják, G Boehm, T S Parker, J Edmond, P A Edwards, A M Fogelman   +5 more
doaj   +1 more source

Post-translational regulation of mevalonate kinase by intermediates of the cholesterol and nonsterol isoprene biosynthetic pathways

Journal of Lipid Research, 1997
To assess the potential for feedback inhibition by isoprene intermediates in the cholesterol and nonsterol isoprene biosynthetic pathway, we expressed human cDNAs encoding mevalonate kinase (MKase), phosphomevalonate kinase (PMKase), and mevalonate ...
D D Hinson, K L Chambliss, M J Toth, R D Tanaka, K M Gibson   +4 more
doaj   +1 more source

HMG-CoA reductase kinase: measurement of activity by methods that preclude interference by inhibitors of HMG-CoA reductase activity or by mevalonate kinase.

Journal of Lipid Research, 1982
Assay of HMG-CoA reductase kinase activity requires HMG-CoA reductase (reductase, E.C. 1.1.1.34) free of associated reductase kinase. Microsomal reductase insensitive to inactivation by Mg-nucleotides alone may be prepared by heating microsomes at 50 ...
H J Harwood, Jr, V W Rodwell
doaj   +1 more source

Investigations of the Evolved Molecular Basis for Terpenoid Biosynthesis in Marine Sponges

Advanced Science, EarlyView.
Confirming and extending a previous observation in another Bubarida sponge, genomic and functional analyses of A. cavernosa reveal that sponges retain the mevalonate pathway and employ single α‐domain T1TSs and UbiA‐type TSs for terpenoid biosynthesis. The absence of T1TSs clustering with other biosynthetic genes tentatively suggests, based on limited ...
Fangyan Chen, Bao Chen, Wenhui Zhang, Guifang Sun, Yao Zhao, Jianpeng Zhang, Baofu Xu   +6 more
wiley   +1 more source

Supramolecular Degraders: An Emerging Paradigm in Targeted Protein Degradation

Advanced Science, EarlyView.
Dynamic supramolecular assembly reshapes targeted protein degradation by coordinating modular degrader construction, delivery, functional integration, and intracellular assembly or activation across proteasomal, endosomal–lysosomal, and autophagy–lysosomal pathways.
Kongjun Liu, Xue Yuan, Yan Zhou, Rui Yuan, Shaocheng Xie, Yong Chen, Peiyi Wang, Jianmei Gao, Qihai Gong   +8 more
wiley   +1 more source

The Cancer Cell Metabolic Reprogramming Remodels the Tumor Microenvironment: Molecular Mechanisms and Therapeutic Strategies

Advanced Science, EarlyView.
This review elucidates how cancer cell metabolic reprogramming—across glucose, lipid, amino acid, and nucleotide pathways—remodels the tumor microenvironment to suppress anti‐tumor immunity and promote immune escape. Targeting these metabolic axes offers promising strategies to overcome immunotherapy resistance and enhance cancer treatment.
Guoqing Xiang, Zhicheng Zhou, Xue Jiang, Zhenguang Du, Yangyang Zhan, Xinli Liu   +5 more
wiley   +1 more source

Upadacitinib Restrains the Pathogenic Fitness of CD4+ T Cells and Aberrant B Cell Programming in Optic Neuritis

Advanced Science, EarlyView.
Single‐cell profiling and functional perturbation reveal coordinated JAK1‐pSTAT3 downstream programs in optic neuritis, including MCL1‐dependent fitness of pathogenic CD4+ Tem cells and glycolysis‐linked, cholesterol‐sensitive B‐cell responses associated with RORA. Upadacitinib disrupts this reciprocal T‐B‐cell circuit and alleviates neuroinflammation,
Gengchen Jiang, Qi Jiang, Lian Han, Yue Peng, Xingyu Liu, Tong Yang, Hao Huang, Jiaying Liu, Yuan Gao, Hui Yang, Wenjun Zou, Zhaohuai Li, Wenru Su   +12 more
wiley   +1 more source