Results 11 to 20 of about 5,767 (207)

A Genetically Encoded Biosensor for Characterizing Transport and Metabolism of Glutarate [PDF]

Advanced Science
Glutarate is a platform chemical with diversified applications. It is also an endogenous metabolite involved in various physiological processes. Deficiency in glutaryl‐CoA dehydrogenase (GcdH) for glutarate catabolism induces the inherited metabolic ...
Kaiyu Gao, Hui Zhang, Yidong Liu, Xianzhi Xu, Wei Liu, Zhaoqi Kang, Rong Xu, Shuang Hou, Ping Han, Chuanjuan Lü, Cuiqing Ma, Ping Xu, Chao Gao   +12 more
doaj   +3 more sources

GCDH Acetylation Orchestrates DNA Damage Response and Autophagy via Mitochondrial ROS to Suppress Hepatocellular Carcinoma Progression [PDF]

Research
Metabolic enzyme dysregulation promotes hepatocellular carcinoma (HCC) progression through metabolic reprogramming and lysine acetylation. Glutaryl-CoA dehydrogenase (GCDH), a key enzyme in lysine metabolism, has been demonstrated to play an essential ...
Wei Tian, Yue Yang, Lili Meng, Chao Ge, Yuqi Liu, Canxue Zhang, Zhihong Huang, Chi Zhang, Hua Tian   +8 more
doaj   +2 more sources

Hepatic CBP/p300 Orchestrate Amino Acid‐Driven Gluconeogenesis through Histone Crotonylation

Advanced Science
The role of amino acid metabolism dysregulation in the development of type 2 diabetes remains elusive. Here, significant associations of human CREBBP/EP300 gene polymorphisms with circulating amino acid and glucose levels are reported. Through integrated
Chunxiang Sheng, Tianjiao Li, Hong Lin, Xiaoqin Ma, Feiye Zhou, Mingzhu Li, Yiru Wang, Shushu Wang, Jialin Tan, Junmin Chen, Yulin Yang, Jianmin Liu, Yufang Bi, Jieli Lu, Xiao Wang, Libin Zhou   +15 more
doaj   +2 more sources

Multimodal imaging of striatal degeneration in Amish patients with glutaryl-CoA dehydrogenase deficiency [PDF]

Brain, 2007
Despite early diagnosis, one-third of Amish infants with glutaryl-CoA dehydrogenase deficiency (GA1) develop striatal lesions that leave them permanently disabled. To better understand mechanisms of striatal degeneration, we retrospectively studied imaging results from 25 Amish GA1 patients homozygous for 1296C>T mutations in GCDH. Asymptomatic infants
Kevin A, Strauss, Jelena, Lazovic, Max, Wintermark, D Holmes, Morton   +3 more
openaire   +4 more sources

Crotonylation and the Risk of Head and Neck Cancer: Insights from a Two-Sample Mendelian Randomization Study [PDF]

International Dental Journal
Background: Head and neck cancer (HNC) remains a major global health burden with limited improvement in survival. Crotonylation has been implicated in tumour biology, but its causal contribution to HNC risk is unclear.
Danya Zhang, Xinjian Zhang, Huiying Li, Anqi Liang, Yelei Liang, Zhiping Wang, Shanwei Shi, Yang Ge, Wenfeng Gao, Xinpeng Liu   +9 more
doaj   +2 more sources

Deglutarylation of glutaryl-CoA dehydrogenase by deacylating enzyme SIRT5 promotes lysine oxidation in mice. [PDF]

J Biol Chem, 2022
Bhatt DP, Mills CA, Anderson KA, Henriques BJ, Lucas TG, Francisco S, Liu J, Ilkayeva OR, Adams AE, Kulkarni SR, Backos DS, Major MB, Grimsrud PA, Gomes CM, Hirschey MD.   +14 more
europepmc   +2 more sources

A low-excretor biochemical phenotype of glutaric aciduria type I: Identification of novel mutations in the glutaryl coa dehydrogenase gene and review of literature from India

Annals of Indian Academy of Neurology, 2020
Muntaj Shaik, Mahesh Kamate, T P Kruthika-Vinod, Anakala Basappa Vedamurthy   +3 more
doaj   +2 more sources

Anaerobic Limonene Metabolism in a Methanogenic Enrichment Involves a Glycine Radical Enzyme. [PDF]

Environ Microbiol
A methanogenic enrichment culture on the natural monoterpene limonene is characterised by a novel glycine radical enzyme for anaerobic hydrocarbon activation, the presence of a microbial loop for necromass utilisation and the presence of a predator of Bacteria and Archaea. ABSTRACT Limonene is a natural monoterpene omnipresent in human environments. It
Lonsing A, Martens GA, Resteu A, Kizina J, Wilkie I, Bahr A, Harder J.   +6 more
europepmc   +2 more sources

Clinical and Metabolic Profile of Glutaric Aciduria Type 1 from North India: Tertiary Centre Experience [PDF]

Journal of Clinical and Diagnostic Research, 2017
Introduction: Glutaric aciduria type 1 is caused by deficiency of glutaryl-CoA dehydogenase leading to accumulation of glutarylcarnitine in blood and excretion of glutaric acid, 3-hyroxyglutaric acid and glutaconic acid in urine.
Ankur Singh, Rajniti Prasad, Seema Kapoor, Om Prakash Mishra   +3 more
doaj   +1 more source

Microencephaly in macrocephaly: Rare report of two siblings with glutaric aciduria type 1

Annals of Movement Disorders, 2021
Glutaric aciduria type 1 is an autosomal recessive disorder caused by mutations in GCDH gene on chromosome 19 leading to the deficiency of glutaryl-CoA dehydrogenase which causes an abnormal metabolism of lysine, hydroxylysine and tryptophan with ...
Ayush Agarwal, Divyani Garg, Sangeeta Agarwal   +2 more
doaj   +1 more source