• <em>Acat1/Soat1</em> knockout extends the mutant <em>Npc1</em> mouse lifespan and ameliorates functional deficiencies in multiple organelles of mutant cells
• 2-methylacetoacetyl-coenzyme A thiolase (beta-ketothiolase) deficiency: one disease - two pathways
• 3-HMG Coenzyme A Lyase Deficiency: Macrocephaly and Left Ventricular Noncompaction with a Novel Mutation
• 3-hydroxy-3-methylglutaryl-CoA lyase deficiency: a case report and literature review
• 3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: Clinical presentation and outcome in a series of 37 patients
• 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: one disease - many faces
• A novel mutation (c.121-13T>A) in the polypyrimidine tract of the splice acceptor site of intron 2 causes exon 3 skipping in mitochondrial acetoacetyl-CoA thiolase gene
• A novel mutation (c.121‑13T&gt;A) in the polypyrimidine tract of the splice acceptor site of intron 2 causes exon 3 skipping in mitochondrial acetoacetyl-CoA thiolase gene
• A Novel Mutation in <em>ACAT1</em> Causing Beta-Ketothiolase Deficiency in a 4-Year-Old Sri Lankan Boy with Metabolic Ketoacidosis
• A Novel Mutation in ACAT1 Causing Beta-Ketothiolase Deficiency in a 4-Year-Old Sri Lankan Boy with Metabolic Ketoacidosis
• A sperm-specific proteome-scale metabolic network model identifies non-glycolytic genes for energy deficiency in asthenozoospermia
• Absence of Nceh1 augments 25-hydroxycholesterol-induced ER stress and apoptosis in macrophages
• Acat1/Soat1 knockout extends the mutant Npc1 mouse lifespan and ameliorates functional deficiencies in multiple organelles of mutant cells
• Analysis of ACAT1 gene variants in a patient with beta-ketothiolase deficiency
• Analysis of ACAT1 gene variants in a patient with β-ketothiolase deficiency
• Analysis of clinical phenotype and ACAT1 gene mutation in a family affected with beta-ketothiolase deficiency
• Anti-apoptotic and Beneficial Metabolic Activities of Resveratrol in Type II Gaucher Disease
• Bayesian multiple hypotheses testing in compositional analysis of untargeted metabolomic data
• Beta-ketothiolase deficiency in a Malaysian infant
• Beta-Ketothiolase Deficiency Presenting with Metabolic Stroke After a Normal Newborn Screen in Two Individuals
• Beta-ketothiolase deficiency: A case with unusual presentation of nonketotic hypoglycemic episodes due to coexistent probable secondary carnitine deficiency
• Beta-ketothiolase deficiency: An unusual cause of recurrent ketoacidosis
• Cholesterol-Lowering Gene Therapy Counteracts the Development of Non-ischemic Cardiomyopathy in Mice
• Clinical analysis and genetic diagnosis of three children with Isoleucine metabolic disorders due to variants of HSD17B10 and ACAT1 genes
• Clinical and genetic analysis of two children with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency
• Clinical and molecular analysis of 6 Chinese patients with isoleucine metabolism defects: identification of 3 novel mutations in the HSD17B10 and ACAT1 gene
• Clinical and Mutational Characterizations of Ten Indian Patients with Beta-Ketothiolase Deficiency
• Clinical presentation and outcome in a series of 32 patients with 2-methylacetoacetyl-coenzyme A thiolase (MAT) deficiency
• Cloning, characterization, and functional analysis of acetyl-CoA C-acetyltransferase and 3-hydroxy-3-methylglutaryl-CoA synthase genes in Santalum album
• Coupled brain and urine spectroscopy - in vivo metabolomic characterization of HMG-CoA lyase deficiency in 5 patients
• Disturbance of redox homeostasis as a contributing underlying pathomechanism of brain and liver alterations in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency
• Dysregulated Free Fatty Acid Receptor 2 Exacerbates Colonic Adenoma Formation in <em>Apc</em> ^{<em>Min/+</em>} Mice: Relation to Metabolism and Gut Microbiota Composition
• Emergent treatment using glucose/insulin infusing for ketoacidosis in T2 deficiency
• Engineering a Balanced Acetyl Coenzyme A Metabolism in <em>Saccharomyces cerevisiae</em> for Lycopene Production through Rational and Evolutionary Engineering
• Engineering Rhodosporidium toruloides for limonene production
• Enhanced acyl-CoA:cholesterol acyltransferase activity increases cholesterol levels on the lipid droplet surface and impairs adipocyte function
• Exon 10 skipping in ACAT1 caused by a novel c.949G&gt;A mutation located at an exonic splice enhancer site
• First report of 3-oxothiolase deficiency in iran
• Formation of Foamy Macrophages by Tuberculous Pleural Effusions Is Triggered by the Interleukin-10/Signal Transducer and Activator of Transcription 3 Axis through ACAT Upregulation
• Genetic, biochemical, and clinical spectrum of patients with mitochondrial trifunctional protein deficiency identified after the introduction of newborn screening in the Netherlands
• Heterozygous Monocarboxylate Transporter 1 (MCT1, SLC16A1) Deficiency as a Cause of Recurrent Ketoacidosis
• How to handle HMG-CoA lyase deficiency and type 1 diabetes? A challenging case report
• Identification of a Novel HADHB Gene Mutation in an Iranian Patient with Mitochondrial Trifunctional Protein Deficiency
• Identification of enzymes involved in oxidation of phenylbutyrate
• Identification of two novel <em>ACAT1</em> variant associated with beta-ketothiolase deficiency in a 9-month-old boy
• Identification of two novel ACAT1 variant associated with beta-ketothiolase deficiency in a 9-month-old boy
• Improvement of 2,3-butanediol yield in Klebsiella pneumoniae by deletion of the pyruvate formate-lyase gene
• Inborn errors of ketone body utilization
• Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria
• Induction of a Proinflammatory Response in Cortical Astrocytes by the Major Metabolites Accumulating in HMG-CoA Lyase Deficiency: the Role of ERK Signaling Pathway in Cytokine Release
• Inflammasome Activation Aggravates Cutaneous Xanthomatosis and Atherosclerosis in ACAT1 (Acyl-CoA Cholesterol Acyltransferase 1) Deficiency in Bone Marrow
• Intestine-specific MTP and global ACAT2 deficiency lowers acute cholesterol absorption with chylomicrons and HDLs
• Iron deficiency triggered transcriptome changes in bread wheat
• Ketoacidotic crisis after vaccination in a girl with beta-ketothiolase deficiency: a case report
• Loss of apoptosis regulator through modulating IAP expression (ARIA) protects blood vessels from atherosclerosis
• Lysine acetylation activates 6-phosphogluconate dehydrogenase to promote tumor growth
• Management and long-term evolution of a patient with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency
• Mechanism of the development of nonalcoholic steatohepatitis after pancreaticoduodenectomy
• Metabolic annotation of 2-ethylhydracrylic acid
• Mitochondrial acetoacetyl-CoA thiolase deficiency: basal ganglia impairment may occur independently of ketoacidosis
• Mitochondrial acetoacetyl-CoA thiolase enzyme deficiency in a 9-month old boy: Atypical urinary metabolic profile with a novel homozygous mutation in ACAT1 gene
• Mitochondrial energy failure in HSD10 disease is due to defective mtDNA transcript processing
• Mitochondrial HMG-CoA Synthase Deficiency: A Cyclic Vomiting Mimic Without Reliable Biochemical Markers
• Mitochondrial trifunctional protein deficiency due to <em>HADHB</em> gene mutation in a Chinese family
• Mitochondrial trifunctional protein deficiency due to HADHB gene mutation in a Chinese family
• Molecular Cloning, Characterization, and Functional Analysis of Acetyl-CoA C-Acetyltransferase and Mevalonate Kinase Genes Involved in Terpene Trilactone Biosynthesis from Ginkgo biloba
• Molecular insights into HSD10 disease: impact of SDR5C1 mutations on the human mitochondrial RNase P complex
• Murine deficiency of peroxisomal L-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol homeostasis
• Mutation update on ACAT1 variants associated with mitochondrial acetoacetyl-CoA thiolase (T2) deficiency
• Myeloid Acyl-CoA:Cholesterol Acyltransferase 1 Deficiency Reduces Lesion Macrophage Content and Suppresses Atherosclerosis Progression
• Neonatal Long-Chain 3-Ketoacyl-CoA Thiolase deficiency: Clinical-biochemical phenotype, sodium-D,L-3-hydroxybutyrate treatment experience and cardiac evaluation using speckle echocardiography
• Newborn screening for 3-hydroxy-3-methylglutaric aciduria using direct analysis in real-time mass spectrometry
• NMDA Receptors and Oxidative Stress Induced by the Major Metabolites Accumulating in HMG Lyase Deficiency Mediate Hypophosphorylation of Cytoskeletal Proteins in Brain From Adolescent Rats: Potential Mechanisms Contributing to the Neuropathology of This D
• Novel role of TLR4 in NAFLD development: Modulation of metabolic enzymes expression
• Oxysterol-binding protein-related protein 1 variants have opposing cholesterol transport activities from the endolysosomes
• Peripheral Neuropathy, Episodic Rhabdomyolysis, and Hypoparathyroidism in a Patient with Mitochondrial Trifunctional Protein Deficiency
• Poly(3-hydroxybutyrate) hyperproduction by a global nitrogen regulator NtrB mutant strain of Paracoccus denitrificans PD1222
• Potentiating the antitumour response of CD8(+) T cells by modulating cholesterol metabolism
• Prevention by L-carnitine of DNA damage induced by 3-hydroxy-3-methylglutaric and 3-methylglutaric acids and experimental evidence of lipid and DNA damage in patients with 3-hydroxy-3-methylglutaric aciduria
• Production of 9,21-dihydroxy-20-methyl-pregna-4-en-3-one from phytosterols in Mycobacterium neoaurum by modifying multiple genes and improving the intracellular environment
• Profile of cardiac lipid metabolism in STZ-induced diabetic mice
• Recent advances in understanding beta-ketothiolase (mitochondrial acetoacetyl-CoA thiolase, T2) deficiency
• Regulation of Pyruvate Formate Lyase-Deficient Klebsiella pneumoniae for Efficient 1,3-Propanediol Bioproduction
• Short-Term Acyl-CoA:Cholesterol Acyltransferase Inhibition, Combined with Apoprotein A1 Overexpression, Promotes Atherosclerosis Inflammation Resolution in Mice
• Single-nucleotide substitution T to A in the polypyrimidine stretch at the splice acceptor site of intron 9 causes exon 10 skipping in the <em>ACAT1</em> gene
• Stabilization of FASN by ACAT1-mediated GNPAT acetylation promotes lipid metabolism and hepatocarcinogenesis
• Structural elucidation of novel biomarkers of known metabolic disorders based on multistage fragmentation mass spectra
• Synthesis of medium-chain length (C6-C10) fuels and chemicals via β-oxidation reversal in Escherichia coli
• The autophagic degradation of cytosolic pools of peroxisomal proteins by a new selective pathway
• The first case in Asia of 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (HSD10 disease) with atypical presentation
• The ketone body β-hydroxybutyrate shifts microglial metabolism and suppresses amyloid-β oligomer-induced inflammation in human microglia
• The management of pregnancy and delivery in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency
• The recent insights into the function of ACAT1: A possible anti-cancer therapeutic target
• Thermo-sensitive mitochondrial trifunctional protein deficiency presenting with episodic myopathy
• TLR4-mediated inflammation promotes foam cell formation of vascular smooth muscle cell by upregulating ACAT1 expression
• TREM2 Regulates Microglial Cholesterol Metabolism upon Chronic Phagocytic Challenge
• Two Infants With Beta-Ketothiolase Deficiency Identified by Newborn Screening in China
• TXNIP regulates myocardial fatty acid oxidation via miR-33a signaling
• Unexplained Tachypneoa and Severe Metabolic Acidosis in a Three-Month-Old Child: A Rare Presentation of Beta-Ketothiolose Deficiency
• Unusual Magnetic Resonance Imaging Findings in 3-Hydroxy-3-Methylglutaryl-Coenzyme A Lyase Deficiency