Disease: HMG CoA synthetase deficiency
- 3-Methylglutaconyl-CoA hydratase, 3-methylcrotonyl-CoA carboxylase and 3-hydroxy-3-methylglutaryl-CoA lyase deficiencies: a coupled enzyme assay useful for their detection
- Ablation of gp78 in liver improves hyperlipidemia and insulin resistance by inhibiting SREBP to decrease lipid biosynthesis
- Airway mesenchymal cell death by mevalonate cascade inhibition: integration of autophagy, unfolded protein response and apoptosis focusing on Bcl2 family proteins
- Cholesterol cell content modulates GTPase activity of G proteins in GH4C1 cell membranes
- Cholesterol synthesis in patients with glutathione deficiency
- Control of sterol metabolism in cultured rat granulosa cells
- Disturbances in the normal regulation of SREBP-sensitive genes in PPAR alpha-deficient mice
- Dysregulated Hepatic Methionine Metabolism Drives Homocysteine Elevation in Diet-Induced Nonalcoholic Fatty Liver Disease
- E3 Ubiquitin Ligase HRD1 Promotes Lung Tumorigenesis by Promoting Sirtuin 2 Ubiquitination and Degradation
- Effects of gamma-linolenic acid supplementation on lipogenesis regulation in pregnant zinc-deficient rat and fetus
- ER-associated ubiquitin ligase HRD1 programs liver metabolism by targeting multiple metabolic enzymes
- Exposure of <em>Aspergillus fumigatus</em> to Atorvastatin Leads to Altered Membrane Permeability and Induction of an Oxidative Stress Response
- Isolation and sequence of the human farnesyl pyrophosphate synthetase cDNA. Coordinate regulation of the mRNAs for farnesyl pyrophosphate synthetase, 3-hydroxy-3-methylglutaryl coenzyme A reductase, and 3-hydroxy-3-methylglutaryl coenzyme A synthase by ph
- Metabolism of leucine in fibroblasts from patients with deficiencies in each of the major catabolic enzymes: branched-chain ketoacid dehydrogenase, isovaleryl-CoA dehydrogenase, 3-methylcrotonyl-CoA carboxylase, 3-methylglutaconyl-CoA hydratase, and 3-hyd
- Mevalonate governs interdependency of ergosterol and siderophore biosyntheses in the fungal pathogen Aspergillus fumigatus
- Myopathies with miscellaneous disorders related to mitochondrial fatty acid oxidation: defective synthesis of ketone body, long-chain fatty acid transport defect, and muscular coenzyme Q10 deficiency
- Regulation of sterol regulatory element-binding proteins by cholesterol flux in CaCo-2 cells
- Role of 26S proteasome and HRD genes in the degradation of 3-hydroxy-3-methylglutaryl-CoA reductase, an integral endoplasmic reticulum membrane protein
- S100A16 promotes acute kidney injury by activating HRD1-induced ubiquitination and degradation of GSK3β and CK1α
- Salicylate activates AMPK and synergizes with metformin to reduce the survival of prostate and lung cancer cells ex vivo through inhibition of de novo lipogenesis
- Selective Screening for Organic Acidurias and Amino Acidopathies in Pakistani Children
- Sequential actions of the AAA-ATPase valosin-containing protein (VCP)/p97 and the proteasome 19 S regulatory particle in sterol-accelerated, endoplasmic reticulum (ER)-associated degradation of 3-hydroxy-3-methylglutaryl-coenzyme A reductase
- Targeting leukostasis for the treatment of early diabetic retinopathy
- Targeting the Mevalonate Pathway Suppresses VHL-Deficient CC-RCC through an HIF-Dependent Mechanism
- The advisory report 'Neonatal screening' from the Health Council of The Netherlands
- The diagnosis of mitochondrial HMG-CoA synthase deficiency
- The effects of triiodothyronine, hydrocortisone and insulin on lipid synthesis by cultured fibroblasts preincubated in a serum-free medium
- The hypolipidemic effects of peptides prepared from Cicer arietinum in ovariectomized rats and HepG2 cells