WebThe glutaryl-CoA so formed serves as a substrate for glutaryl-CoA dehydrogenase (GCDH: EC 1.3.99.7), which is located downstream of hE1a on the L-lysine degradative pathway and produces glutaconyl ... WebGlutaryl-CoA dehydrogenase (GCDH) deficiency is an autosomal recessive disease with an estimated overall prevalence of 1 in 100 000 newborns. Biochemically, the …
Deglutarylation of glutaryl-CoA dehydrogenase by …
WebFeb 17, 2024 · Glutaryl-CoA dehydrogenase (GCDH; EC 1.3.8.6) is an acyl dehydrogenase involved in the metabolism of lysine, hydroxylysine, and … WebApr 1, 2024 · Further downstream in this degradation pathway, glutaryl-CoA is converted into crotonyl-CoA by glutaryl-CoA dehydrogenase (GCDH), which is exclusive to the … cyclone hot water heater troubleshooting
Exploring genotype-phenotype correlations in glutaric aciduria
Lysine restriction inhibits colon cancer cell growth7,8, while acetyl-CoA generated from lysine catabolism drives liver metastasis of colorectal tumours36. To directly assess the importance of the lysine catabolism pathway for melanoma cell viability, we assayed whether individual components of this pathway affect … See more To identify possible mechanisms underlying cell death, we monitored changes in gene expression following GCDH KD in melanoma cells. RNA-sequencing (RNA-seq) analysis followed by pathway analysis … See more We next assessed whether genetic GCDH inhibition would impact melanoma growth in vivo. Inducible inactivation of GCDH was established in A375 cells using doxycycline (Dox) … See more Given NRF2 upregulation in the brain of GCDH KO mice fed a high lysine diet27, and that NRF2 can be linked with ATF3 and ATF4 transcription29,32,40,41, we asked whether … See more To monitor potential changes in NRF2 stability following GCDH KD, we performed cycloheximide chase assays in A375 melanoma cells. NRF2 half-life increased following GCDH KD in A375 relative to control … See more WebGlutaric aciduria type 1 (GA1) is a rare neurometabolic disease caused by pathogenic variants in the gene encoding the enzyme glutaryl-CoA dehydrogenase (GCDH). We performed an extensive literature search to collect data on GA1 patients, together with unpublished cases, to provide an up-to-date gene … WebJun 28, 2024 · enzyme glutaryl-CoA dehydrogenase (GCDH). We show increased GCDH glutarylation when glutaryl-CoA production is stimulated by lysine catabolism. Our data reveal glutarylation of GCDH impacts its function, ultimately decreasing lysine oxidation. We then demonstrate the ability of SIRT5 to deglutarylate GCDH, restoring its enzymatic … cyclone hoover