"Acid lipase disease Acid lipase disease or deficiency is a name used to describe two related disorders of fatty acid metabolism. Acid lipase disease occurs when the enzyme lysosomal acid lipase that is needed to break down certain fats that are normally digested by the body is lacking or missing .This results in the toxic buildup of these fats in the body’s cells and tissues. These fatty substances, called lipids, include waxes, oils, and cholesterol. Three rare lipid storage diseases are caused by the deficiency of the enzyme lysosomal acid lipase: The prevalence of Lysosomal acid lipase deficiency is unknown"
"water. Some fatty acids that are set free by the digestion of fats are called essential because they cannot be synthesized in the body from simpler constituents. There are two essential fatty acids (EFAs) in human nutrition: alpha-linolenic acid (an omega-3 fatty acid) and linoleic acid (an omega-6 fatty acid). Other lipids needed by the body can be synthesized from these and other fats. Fats and other lipids are broken down in the body by enzymes called lipases produced in the pancreas. Fats and oils are categorized according to the number and bonding of the carbon atoms in the aliphatic"
"is also present in lipases of prokaryotic origin and in lecithin-cholesterol acyltransferase () (LCAT), which catalyzes fatty acid transfer between phosphatidylcholine and cholesterol. ""Pancreatic lipase"", also known as ""pancreatic triacylglycerol lipase"" or ""steapsin"", is an enzyme secreted from the pancreas. As the primary lipase enzyme that hydrolyzes (breaks down) dietary fat molecules in the human digestive system, it is one of the main digestive enzymes, converting triglyceride substrates found in ingested oils to monoglycerides and free fatty acids. Bile salts secreted from the liver and stored in gallbladder are released into the duodenum, where they coat and emulsify large fat"
"pancreas. Measured serum lipase values may vary depending on the method of analysis. Lipase can also assist in the breakdown of fats into lipids in those undergoing pancreatic enzyme replacement therapy (PERT). It is a key component in Sollpura (Liprotamase). 25. Gulzar, Bio-degradation of hydrocarbons using different bacterial and fungal species. Published in international conference on biotechnology and neurosciences. CUSAT (cochin university of science and technology), 2003 Lipase A lipase (, ) is any enzyme that catalyzes the hydrolysis of fats (lipids). Lipases are a subclass of the esterases. Lipases perform essential roles in digestion, transport and processing of dietary"
"causing more serious complications. ACADM ACADM (acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain) is a gene that provides instructions for making an enzyme called acyl-coenzyme A dehydrogenase that is important for breaking down (degrading) a certain group of fats called medium-chain fatty acids. These fatty acids are found in foods such as milk and certain oils, and they are also stored in the body's fat tissue. Medium-chain fatty acids are also produced when larger fatty acids are degraded. The acyl-coenzyme A dehydrogenase for medium-chain fatty acids (ACADM) enzyme is essential for converting these particular fatty acids to energy, especially"
"cleaved, creating a cascade of activating enzymes: enteropeptidase activates the proenzyme trypsinogen by cleaving it to form trypsin. The free trypsin then cleaves the rest of the trypsinogen, as well as chymotrypsinogen to its active form chymotrypsin. The pancreas secretes substances which help in the digestion of starch and other carbohydrates, proteins and fats. Proteases, the enzymes involved in the digestion of proteins, include trypsinogen and chymotrypsinogen. The enzyme involved in the digestion of fats is lipase. Amylase, also secreted by the pancreas, breaks down starch (amylum) and other carbohydrates. The pancreas also secretes phospholipase A2, lysophospholipase, and cholesterol esterase."
"enzyme is so efficient that the rate for short chain fatty acids is equivalent to that of diffusion-controlled reactions. ECH catalyzes the second step (hydratation) in the breakdown of fatty acids (β-oxidation). Fatty acid metabolism is how human bodies turn fats into energy. Fats in foods are generally in the form of triglycerols. These must be broken down in order for the fats to pass into human bodies. When that happens, three fatty acids are released. ECH is used in β-oxidation to add a hydroxyl group and a proton to the unsaturated β-carbon on a fatty-acyl CoA. ECH functions by"
"acid and other digestive enzymes to break down food. Food moves from the stomach into the small intestine. The first part of the small intestine is the duodenum. As food moves through the duodenum, it mixes with bile, a fluid that neutralizes stomach acid and emulsifies fat. The pancreas releases enzymes that aid in digestion so that nutrients can be broken down and pass through the intestinal mucosa into the blood and travel to the rest of the body. The pancreas doesn't produce starch processing enzymes because cats don't eat a diet high in carbohydrates. Since the cat digests low"
"ACADM ACADM (acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain) is a gene that provides instructions for making an enzyme called acyl-coenzyme A dehydrogenase that is important for breaking down (degrading) a certain group of fats called medium-chain fatty acids. These fatty acids are found in foods such as milk and certain oils, and they are also stored in the body's fat tissue. Medium-chain fatty acids are also produced when larger fatty acids are degraded. The acyl-coenzyme A dehydrogenase for medium-chain fatty acids (ACADM) enzyme is essential for converting these particular fatty acids to energy, especially during periods without food"
"chromosome 16q24. The gene encodes the enzyme malonyl-CoA decarboxylase. Within cells, this enzyme helps regulate the formation and breakdown of a certain group of fats called fatty acids. Many tissues, including heart muscle, use fatty acids as a major source of energy. Mutations in the ""MLYCD"" gene reduce or eliminate the function of malonyl-CoA decarboxylase. A lack of this enzyme disrupts the normal balance of fatty acid formation and breakdown. As a result, fatty acids cannot be converted to energy, which can lead to characteristic features of this disorder, such as low blood sugar and cardiomyopathy. By-products of fatty acid"
"Fatty acid amide hydrolase Fatty acid amide hydrolase or FAAH (, oleamide hydrolase, anandamide amidohydrolase) is a member of the serine hydrolase family of enzymes. It was first shown to break down anandamide in 1993. In humans, it is encoded by the gene ""FAAH"". FAAH is an integral membrane hydrolase with a single ""N""-terminal transmembrane domain. ""In vitro"", FAAH has esterase and amidase activity. ""In vivo"", FAAH is the principal catabolic enzyme for a class of bioactive lipids called the fatty acid amides (FAAs). Members of the FAAs include: ""FAAH"" knockout mice display highly elevated (>15-fold) levels of ""N""-acylethanolamines and"
"proteins into polypeptides that are then broken down by various exopeptidases and dipeptidases into amino acids. The digestive enzymes however are mostly secreted as their inactive precursors, the zymogens. For example, trypsin is secreted by pancreas in the form of trypsinogen, which is activated in the duodenum by enterokinase to form trypsin. Trypsin then cleaves proteins to smaller polypeptides. Digestion of some fats can begin in the mouth where lingual lipase breaks down some short chain lipids into diglycerides. However fats are mainly digested in the small intestine. The presence of fat in the small intestine produces hormones that stimulate"
"perform a different function. This enzyme complex is required to metabolize a group of fats called long-chain fatty acids. These fatty acids are stored in the body's fat tissues and are a major source of energy for the heart and muscles. During periods of fasting, fatty acids are also an important energy source for the liver and other tissues. Mutations in the HADHA or HADHB genes that cause mitochondrial trifunctional protein deficiency disrupt all functions of this enzyme complex. Without enough of this enzyme complex, long-chain fatty acids cannot be metabolized. As a result, these fatty acids are not converted"
"in fatty tissues, such as adipose, cardiac, and muscle. Lipoprotein lipase is downregulated by high levels of insulin, and upregulated by high levels of glucagon and adrenaline. Pectinases, also called pectolytic enzymes, are a class of exoenzymes that are involved in the breakdown of pectic substances, most notably pectin. Pectinases can be classified into two different groups based on their action against the galacturonan backbone of pectin: de-esterifying and depolymerizing. These exoenzymes can be found in both plants and microbial organisms including fungi and bacteria. Pectinases are most often used to break down the pectic elements found in plants and"
"enables it to cross-link antigens, allowing use for precipitation assays, cellular aggregation via surface antigens, or rosetting assays. The following three genes encode identical human pepsinogen A enzymes: A fourth human gene encodes gastricsin also known as pepsinogen C: Pepsin Pepsin is an endopeptidase that breaks down proteins into smaller peptides (that is, a protease). It is produced in the stomach and is one of the main digestive enzymes in the digestive systems of humans and many other animals, where it helps digest the proteins in food. Pepsin has a three-dimensional structure, of which one or more polypeptide chains twist"
"the body, which becomes a nice intermediate for other reactions such as glycolysis. Lipases hydrolyze glycerides. Glycosidases cleave sugar molecules off carbohydrates and peptidases hydrolyze peptide bonds. Nucleosidases hydrolyze the bonds of nucleotides. Hydrolase enzymes are important for the body because they have degradative properties. In lipids, lipases contribute to the breakdown of fats and lipoproteins and other larger molecules into smaller molecules like fatty acids and glycerol. Fatty acids and other small molecules are used for synthesis and as a source of energy. In biochemistry, a hydrolase is an enzyme that catalyzes the hydrolysis of a chemical bond. For"
"the catalytic activity of gastric lipase. At the N-terminal, Lys4 is necessary for the enzyme to bind at lipid-water interfaces. Pancreatic lipase is the most important lipolytic enzyme in the gastrointestinal tract and is essential for fat digestion. Pancreatic lipase is secreted from acinar cells in the pancreas and its secretion, with the pancreatic juice to the small intestine, is stimulated by hormones. These hormones are induced in the stomach by hydrolysed products in gastric digestion. The pancreatic lipase is secreted to the small intestine where it is most active, at pH 7-7,5. Pancreatic lipase hydrolyses triglycerides and diglycerides by"
"an enzyme found in tears, saliva and egg whites that digests the coating of some bacteria; the structure was solved by a group led by David Chilton Phillips and published in 1965. This high-resolution structure of lysozyme marked the beginning of the field of structural biology and the effort to understand how enzymes work at an atomic level of detail. The term ""metabolism"" is derived from the Greek Μεταβολισμός – Metabolismos for ""change"", or ""overthrow"". The history of the scientific study of metabolism spans 800 years. The earliest of all metabolic studies began during the early thirteenth century (1213-1288) by"
"Biotinidase Biotinidase (, ""amidohydrolase biotinidase"", ""BTD"") is an enzyme that in humans is encoded by the ""BTD"" gene. This enzyme allows the body to use and to recycle the B vitamin biotin, sometimes called vitamin H. Biotinidase extracts biotin from food because the body needs biotin in its free, unattached form. This enzyme also recycles biotin from enzymes in the body that use it as a helper component in order to function. These enzymes, known as carboxylases, are important in the processing of fats, carbohydrates, and proteins. Biotin is attached to these carboxylase enzymes through an amino acid (the building"
"droplets into smaller droplets, thus increasing the overall surface area of the fat, which allows the lipase to break apart the fat more effectively. The resulting monomers (2 free fatty acids and one 2-monoacylglycerol) are then moved by way of peristalsis along the small intestine to be absorbed into the lymphatic system by a specialized vessel called a lacteal. This protein belongs to the pancreatic lipase family. Unlike some pancreatic enzymes that are activated by proteolytic cleavage (e.g., trypsinogen), pancreatic lipase is secreted in its final form. However, it becomes efficient only in the presence of colipase in the duodenum."
"Deamination Deamination is the removal of an amino group from a molecule. Enzymes that catalyse this reaction are called deaminases. In the human body, deamination takes place primarily in the liver, however glutamate is also deaminated in the kidneys. In situations of excess protein intake, deamination is used to break down amino acids for energy. The amino group is removed from the amino acid and converted to ammonia. The rest of the amino acid is made up of mostly carbon and hydrogen, and is recycled or oxidized for energy. Ammonia is toxic to the human system, and enzymes convert it"
"intestinal wall. In the epithelial cells, fatty acids are packaged and transported to the rest of the body. Digestion is the first step to lipid metabolism, and it is the process of breaking the triglycerides down into smaller monoglyceride units with the help of lipase enzymes. Digestion of fats begin in the mouth through chemical digestion by lingual lipase. Ingested cholesterol is not broken down by the lipases and stays intact until it enters the epithelium cells of small intestine. Lipids then continue to the stomach where chemical digestion continues by gastric lipase and mechanical digestion begins (Peristalsis). The majority"
"fats to some degree though is not as efficient as the pancreatic lipase. The pylorus, the lowest section of the stomach which attaches to the duodenum via the pyloric canal, contains countless glands which secrete digestive enzymes including gastrin. After an hour or two, a thick semi-liquid called chyme is produced. When the pyloric sphincter, or valve opens, chyme enters the duodenum where it mixes further with digestive enzymes from the pancreas, and then passes through the small intestine, where digestion continues. When the chyme is fully digested, it is absorbed into the blood. 95% of absorption of nutrients occurs"
"Bile salt-dependent lipase Bile salt-dependent lipase (or BSDL), also known as carboxyl ester lipase (or CEL) is an enzyme produced by the adult pancreas and aids in the digestion of fats. Bile salt-stimulated lipase (or BSSL) is an equivalent enzyme found within breast milk. BSDL has been found in the pancreatic secretions of all species in which it has been looked for. BSSL, originally discovered in the milk of humans and various other primates, has since been found in the milk of many animals including dogs, cats, rats, and rabbits. More than 95% of the fat present in human milk"
"(fasting). The ACADM enzyme functions in mitochondria, the energy-producing centers within cells. It is found in the mitochondria of several types of tissues, particularly the liver. The ACADM gene is located on the short (p) arm of chromosome 1 at position 31, from base pair 75,902,302 to base pair 75,941,203. The protein encoded by the ACADM gene is 50.8 kDa in size, and composed of 454 amino acids. The LCAD enzyme catalyzes most of fatty acid beta-oxidation by forming a C2-C3 trans-double bond in the fatty acid. MCAD works on long-chain fatty acids, typically between C4 and C12-acylCoA. Fatty acid"
"of lipid digestion and absorption, however, occurs once the fats reach the small intestines. Chemicals from the pancreas (pancreatic lipase family and Bile salt-dependent lipase) are secreted into the small intestines to help breakdown the triglycerides, along with further mechanical digestion, until they are individual fatty acid units able to be absorbed into the small intestine's epithelial cells. It is the pancreatic lipase that is responsible for signaling for the hydrolysis of the triglycerides into separate free fatty acids and glycerol units. The second step in lipid metabolism is the absorption of fats. Absorption of fats occurs only in the"
"Lipid metabolism Lipid metabolism is the synthesis and degradation of lipids in cells, involving the break down or storage of fats for energy. These fats are obtained from consuming food and absorbing them or they are synthesized by an animal's liver. Lipogenesis is the process of synthesizing these fats. The majority of lipids found in the human body from ingesting food are triglycerides and cholesterol. Other types of lipids found in the body are fatty acids and membrane Lipids. Lipid metabolism is often considered the digestion and absorption process of dietary fat; however, there are two ways organisms can use"
"plant-derived products. Discovered in 1836, pepsin was one of the first enzymes to be classified as an exoenzyme. The enzyme is first made in the inactive form, pepsinogen by chief cells in the lining of the stomach. With an impulse from the vagus nerve, pepsinogen is secreted into the stomach, where it mixes with hydrochloric acid to form pepsin. Once active, pepsin works to break down proteins in foods such as dairy, meat, and eggs. Pepsin works best at the pH of gastric acid, 1.5 to 2.5, and is deactivated when the acid is neutralized to a pH of 7."
"(VLDL). VLDL particles are secreted directly into blood, where they function to deliver the endogenously derived lipids to peripheral tissues. Fatty acid synthesis starts with acetyl-CoA and builds up by the addition of two-carbon units. Fatty acid synthesis occurs in the cytoplasm of cells, in contrast to the Fatty acid degradation (oxidation), which occurs in the mitochondria. Many of the enzymes for the fatty acid synthesis are organized into a multienzyme complex called fatty acid synthase. The major sites of fatty acid synthesis are adipose tissue and the liver. Triglycerides are synthesized by esterification of fatty acids to glycerol. Fatty"
"start at acetyl-CoA when fat is being broken down for energy if there is little or no glucose available. The energy yield of the complete oxidation of the fatty acid palmitate is 106 ATP. Unsaturated and odd-chain fatty acids require additional enzymatic steps for degradation. Most of the fat found in food is in the form of triglycerides, cholesterol, and phospholipids. Some dietary fat is necessary to facilitate absorption of fat-soluble vitamins (A, D, E, and K) and carotenoids. Humans and other mammals have a dietary requirement for certain essential fatty acids, such as linoleic acid (an omega-6 fatty acid)"
"from the breakdown of fats from adipose tissue. Fats are broken down into glycerol and free fatty acids, with the glycerol being turned into glucose in the liver via the gluconeogenesis pathway. When even the glucose made from glycerol reserves start declining, the liver starts producing ketone bodies. Ketone bodies are short-chain derivatives of the free fatty acids mentioned in the previous paragraph, and can cross the blood–brain barrier, meaning they can be used by the brain as an alternative metabolic fuel. Fatty acids can be used directly as an energy source by most tissues in the body. After the"
"Delta 6 desaturase Delta 6 desaturase (D6D or Δ-6-desaturase) is a desaturase enzyme that converts between types of fatty acids (termed ""6"" after omega-6 fatty acids), which are essential nutrients in the human body. The enzyme is molecularly identical across all living things (preserved across Kingdom (biology)) it is present in animals, plants, and cyanobacteria. D6D is one of the 3 fatty acid desaturases present in humans along with Δ-5 and Δ-9, named so because it was thought to convert only omega-6 fatty acids, but actually converts some others also, and is obligatory to build the longer chain omega-3 fatty"
"use enzymes to speed up chemical reactions: enzymes in biological washing powders break down protein, starch or fat stains on clothes, and enzymes in meat tenderizer break down proteins into smaller molecules, making the meat easier to chew. By the late 17th and early 18th centuries, the digestion of meat by stomach secretions and the conversion of starch to sugars by plant extracts and saliva were known but the mechanisms by which these occurred had not been identified. French chemist Anselme Payen was the first to discover an enzyme, diastase, in 1833. A few decades later, when studying the fermentation"