Journal of Inherited Metabolic Disease: The Journal of Inherited Metabolic Disease is a peer-reviewed medical journal covering inherited metabolic disorders. It was established in 1978 and is the official journal of the Society for the Study of Inborn Errors of Metabolism.Lipotoxicity: Lipotoxicity is a metabolic syndrome that results from the accumulation of lipid intermediates in non-adipose tissue, leading to cellular dysfunction and death. The tissues normally affected include the kidneys, liver, heart and skeletal muscle.Lipid droplet: Lipid droplets, also referred to as lipid bodies, oil bodies or adiposomes, are lipid-rich cellular organelles that regulate the storage and hydrolysis of neutral lipids and are found largely in the adipose tissue.Mobilization and cellular uptake of stored fats and triacylglycerol (with Animation) They also serve as a reservoir for cholesterol and acyl-glycerols for membrane formation and maintenance.Lysinuric protein intoleranceNeutral lipid storage disease: Neutral lipid storage disease (also known as Chanarin–Dorfman syndrome) is an autosomal recessive disorder characterized by accumulation of triglycerides in the cytoplasm of leukocytes, muscle, liver, fibroblasts, and other tissues.Freedberg, et al.UDP-3-O-(3-hydroxymyristoyl)glucosamine N-acyltransferase: UDP-3-O-(3-hydroxymyristoyl)glucosamine N-acyltransferase (, UDP-3-O-acyl-glucosamine N-acyltransferase, UDP-3-O-(R-3-hydroxymyristoyl)-glucosamine N-acyltransferase, acyltransferase LpxD, acyl-ACP:UDP-3-O-(3-hydroxyacyl)-GlcN N-acyltransferase, firA (gene), lpxD (gene)) is an enzyme with system name (3R)-3-hydroxymyristoyl-(acyl-carrier protein):UDP-3-O-((3R)-3-hydroxymyristoyl)-alpha-D-glucosamine N-acetyltransferase. This enzyme catalyses the following chemical reactionHypolipoproteinemiaLiver sinusoid: A liver sinusoid is a type of sinusoidal blood vessel (with fenestrated, discontinuous endothelium) that serves as a location for the oxygen-rich blood from the hepatic artery and the nutrient-rich blood from the portal vein.SIU SOM Histology GIIndex of energy articles: This is an index of energy articles.Heptadecanoic acidModel lipid bilayer: A model lipid bilayer is any bilayer assembled in vitro, as opposed to the bilayer of natural cell membranes or covering various sub-cellular structures like the nucleus. A model bilayer can be made with either synthetic or natural lipids.TriglycerideCholesterolHyperammonemiaLipid peroxidationHyperphenylalaninemia: (also includes non-classic PKU)Smith–Lemli–Opitz syndromeGlucose transporterPhospholipidSilent mutation: Silent mutations are mutations in DNA that do not significantly alter the phenotype of the organism in which they occur. Silent mutations can occur in non-coding regions (outside of genes or within introns), or they may occur within exons.Systemic primary carnitine deficiencyAutorefractor: An autorefractor or automated refractor is a computer-controlled machine used during an eye examination to provide an objective measurement of a person's refractive error and prescription for glasses or contact lenses. This is achieved by measuring how light is changed as it enters a person's eye.Adipose tissue macrophages: Adipose tissue macrophages (abbr. ATMs) comprise tissue resident macrophages present in adipose tissue.Low-sulfur diet: A low-sulfur diet is a diet with reduced sulfur content. Sulfur containing compounds may also be referred to as thiols or mercaptans.Chronic mucocutaneous candidiasisBeano (dietary supplement): Beano is an enzyme-based dietary supplement that is used to reduce gas in the digestive tract, thereby improving digestion and reducing bloating, discomfort, and flatulence caused by gas. It contains the enzyme alpha-galactosidase.Coles PhillipsFabry disease: (ILDS E75.25)Flux (metabolism): Flux, or metabolic flux is the rate of turnover of molecules through a metabolic pathway. Flux is regulated by the enzymes involved in a pathway.Giralang, Australian Capital TerritoryOrnithine transcarbamylase: Ornithine transcarbamylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme that catalyzes the reaction between carbamoyl phosphate (CP) and ornithine (Orn) to form citrulline (Cit) and phosphate (Pi). In plants and microbes, OTC is involved in arginine (Arg) biosynthesis, whereas in mammals it is located in the mitochondria and is part of the urea cycle.Matrix model: == Mathematics and physics ==Argininosuccinic acidTable of standard reduction potentials for half-reactions important in biochemistry: The values below are standard reduction potentials for half-reactions measured at 25°C, 1 atmosphere and a pH of 7 in aqueous solution.Insulin signal transduction pathway and regulation of blood glucose: The insulin transduction pathway is an important biochemical pathway beginning at the cellular level affecting homeostasis. This pathway is also influenced by fed versus fasting states, stress levels, and a variety of other hormones.HypophosphatasiaBlood glucose monitoring: Blood glucose monitoring is a way of testing the concentration of glucose in the blood (glycemia). Particularly important in the care of diabetes mellitus, a blood glucose test is performed by piercing the skin (typically, on the finger) to draw blood, then applying the blood to a chemically active disposable 'test-strip'.Animal fatMethylmalonic acidVery low-density lipoprotein: Very-low-density lipoprotein (VLDL) is a type of lipoprotein made by the liver. VLDL is one of the five major groups of lipoproteins (chylomicrons, VLDL, low-density lipoprotein, intermediate-density lipoprotein, high-density lipoprotein) that enable fats and cholesterol to move within the water-based solution of the bloodstream.Temporal analysis of products: Temporal Analysis of Products (TAP), (TAP-2), (TAP-3) is an experimental technique for studyingPrescription cascade: Prescription cascade refers to the process whereby the side effects of drugs are misdiagnosed as symptoms of another problem resulting in further prescriptions and further side effects and unanticipated drug interactions. This may lead to further misdiagnoses and further symptoms.Isovaleric acidemiaSpin–lattice relaxation in the rotating frame: Spin–lattice relaxation in the rotating frame is the mechanism by which Mxy, the transverse component of the magnetization vector, exponentially decays towards its equilibrium value of zero, under the influence of a radio frequency (RF) field in nuclear magnetic resonance (NMR) and magnetic resonance imaging (MRI). It is characterized by the spin–lattice relaxation time constant in the rotating frame, T1ρ.Lipolysis: Lipolysis is the breakdown of lipids and involves hydrolysis of triglycerides into glycerol and free fatty acids. The following hormones induce lipolysis: epinephrine, norepinephrine, ghrelin, growth hormone, testosterone, and cortisol.HydroxocobalaminMethylmalonyl-CoAList of MeSH codes (D12.776.930): This is a sub-part (transcription factors only) of List of MeSH codes (D12.776), itself a part of the list of the "D" codes for MeSH.Phenotype microarray: The phenotype microarray approach is a technology for high-throughput phenotyping of cells.SotolonEgg lecithinAtomic mass: right |thumb|200px|Stylized [[lithium-7 atom: 3 protons, 4 neutrons, & 3 electrons (total electrons are ~1/4300th of the mass of the nucleus). It has a mass of 7.Arginine:glycine amidinotransferaseGene signature: A gene signature is a group of genes in a cell whose combined expression patternItadani H, Mizuarai S, Kotani H. Can systems biology understand pathway activation?Erythropoietic porphyriaGlutaryl-CoA dehydrogenase: Glutaryl-CoA dehydrogenase (GCDH) is an enzyme encoded by the GCDH gene on chromosome 19. The protein belongs to the acyl-CoA dehydrogenase family (ACD).Orphan receptor: An orphan receptor is an apparent receptor that has a similar structure to other identified receptors but whose endogenous ligand has not yet been identified. If a ligand for an orphan receptor is later discovered, the receptor is referred to as an "adopted orphan".Mature messenger RNA: Mature messenger RNA, often abbreviated as mature mRNA is a eukaryotic RNA transcript that has been spliced and processed and is ready for translation in the course of protein synthesis. Unlike the eukaryotic RNA immediately after transcription known as precursor messenger RNA, it consists exclusively of exons, with all introns removed.Argininemia: Argininemia, also called arginase deficiency, is an autosomal recessive urea cycle disorder where a deficiency of the enzyme arginase causes a buildup of arginine and ammonia in the blood.Symmetry element: A symmetry element is a point of reference about which symmetry operations can take place. In particular, symmetry elements can be centers of inversion, axes of rotation and mirror planes.Medium-chain acyl-coenzyme A dehydrogenase deficiencyBile acid malabsorptionLipoprotein lipase: Lipoprotein lipase (LPL) () is a member of the lipase gene family, which includes pancreatic lipase, hepatic lipase, and endothelial lipase. It is a water-soluble enzyme that hydrolyzes triglycerides in lipoproteins, such as those found in chylomicrons and very low-density lipoproteins (VLDL), into two free fatty acids and one monoacylglycerol molecule.Classification of obesity: Obesity is a medical condition in which excess body fat has accumulated to the extent that it has an adverse effect on health.WHO 2000 p.Fatty liverGas chromatography–mass spectrometry: right|300 px|Example of a GC-MS instrument|thumb