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3-hydroxyacyl-coenzyme A Dehydrogenase Deficiency
3-hydroxyacyl-coenzyme A dehydrogenase deficiency is a rare condition that prevents the body from converting certain fats to energy, particularly during fasting. Normally, through a process called fatty acid oxidation, several enzymes work in a step-wise fashion to metabolize fats and convert them to energy. People with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency have inadequate levels of an enzyme required for a step that metabolizes groups of fats called medium chain fatty acids and short chain fatty acids; for this reason this disorder is sometimes called medium- and short-chain 3-hydroxyacyl-coenzyme A dehydrogenase (M/SCHAD) deficiency. Signs and symptoms Typically, initial signs and symptoms of this disorder occur during infancy or early childhood and can include poor appetite, vomiting, diarrhea, lethargy, hypoglycemia, hypotonia, liver problems, and hyperinsulinism (high levels of insulin). Insulin controls the amount of sugar that moves from the blood into cells for c ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Fatty Acid Oxidation
Fatty is a derogatory term for someone who is Obesity, obese. It may refer also to: People * Mai Fatty, Gambian politician * Roscoe Arbuckle (1887–1933), American actor and comedian * Fatty Briody (1858–1903), American Major League Baseball player * Fatty D (April Fores), American pornographic actress * Bob Fothergill (1897–1938), American Major League Baseball outfielder * William Foulke (footballer) (1874–1916), English cricketer and footballer * Fatty George (1927–1982), Austrian jazz musician * Richard Lamb (1907–1974), Australian racing cyclist * Fatty Lawrence (1903–1976), college gridiron football player * W. T. McLain (1885–1938), college gridiron football player, lawyer, and politician * Charles H. Smith (American football), University of Michigan football player in 1893–1894 * Roland Taylor (1946–2017), retired American Basketball Association and National Basketball Association player * Paul Vautin (born 1959), Australian former rugby league football ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Medium Chain Fatty Acid
Medium-chain triglycerides (MCTs) are triglycerides with two or three fatty acids having an aliphatic tail of 6–12 carbon atoms, i.e. medium-chain fatty acids (MCFAs). Rich food sources for commercial extraction of MCTs include palm kernel oil and coconut oil. Sources of MCTs MCTs are found in palm kernel oil and coconut oil and can be separated by fractionation. They can also be produced by interesterification. Retail MCT powder is MCT oil embedded in starch and thus contains carbohydrates in addition to fats. It is manufactured by spray drying. List of MCFAs With regard to MCFAs, apart from the above listed straight chain (unbranched chain) fatty acids, side chain (branched chain) fatty acids also exist. Applications Calorie restriction A 2020 systematic review and meta-analysis by ''Critical Reviews in Food Science and Nutrition'' supported evidence that MCT decreases subsequent energy intake, but does not appear to affect appetite. Dietary relevance Molecular w ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Short Chain Fatty Acid
Short-chain fatty acids (SCFAs) are fatty acids with fewer than six carbon atoms. Derived from intestinal microbial fermentation of indigestible foods, SCFAs are the main energy source of colonocytes, making them crucial to gastrointestinal health. SCFAs all possess varying degrees of water solubility, which distinguishes them from longer chain fatty acids that are immiscible. List of SCFAs Functions SCFAs are produced when dietary fiber is fermented in the colon. Macronutrient composition (carbohydrate, protein, or fat) of diets affects circulating SCFAs. Acetate, propionate, and butyrate are the three most common SCFAs. SCFAs and medium-chain fatty acids are primarily absorbed through the portal vein during lipid digestion, while long-chain fatty acids are packed into chylomicrons, enter lymphatic capillaries, then transfer to the blood at the subclavian vein. SCFAs have diverse physiological roles in body functions. They can affect the production of lipids, energy and vit ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lethargy
Lethargy is a state of tiredness, sleepiness, weariness, fatigue, sluggishness or lack of energy. It can be accompanied by depression, decreased motivation, or apathy. Lethargy can be a normal response to inadequate sleep, overexertion, overworking, stress, lack of exercise, improper nutrition, boredom, or a symptom of an underlying illness or a disorder. It may also be a side-effect of medication or caused by an interaction between medications or medication(s) and alcohol. It may also be an altered level of consciousness. When part of a normal response, lethargy often resolves with rest, adequate sleep, decreased stress, physical exercise, and good nutrition. Lethargy's symptoms however can last days or even months, so it can be a sign of a recent underlying illness or infection if it doesn't resolve normally. See also * Clinical depression * Encephalitis lethargica * Sleep apnea * Sleep deprivation * Somnolence Somnolence (alternatively sleepiness or drowsiness) is a ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hypoglycemia
Hypoglycemia, also called low blood sugar, is a fall in blood sugar to levels below normal, typically below 70 mg/dL (3.9 mmol/L). Whipple's triad is used to properly identify hypoglycemic episodes. It is defined as blood glucose below 70 mg/dL (3.9 mmol/L), symptoms associated with hypoglycemia, and resolution of symptoms when blood sugar returns to normal. Hypoglycemia may result in headache, tiredness, clumsiness, trouble talking, confusion, fast heart rate, sweating, shakiness, nervousness, hunger, loss of consciousness, seizures, or death. Symptoms typically come on quickly. The most common cause of hypoglycemia is medications used to treat diabetes such as insulin, sulfonylureas, and biguanides. Risk is greater in diabetics who have eaten less than usual, recently exercised, or consumed alcohol. Other causes of hypoglycemia include severe illness, sepsis, kidney failure, liver disease, hormone deficiency, tumors such as insulinomas or non-B cell tumo ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hypotonia
Hypotonia is a state of low muscle tone (the amount of tension or resistance to stretch in a muscle), often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but a potential manifestation of many different diseases and disorders that affect motor nerve control by the brain or muscle strength. Hypotonia is a lack of resistance to passive movement, whereas muscle weakness results in impaired active movement. Central hypotonia originates from the central nervous system, while peripheral hypotonia is related to problems within the spinal cord, peripheral nerves and/or skeletal muscles. Severe hypotonia in infancy is commonly known as floppy baby syndrome. Recognizing hypotonia, even in early infancy, is usually relatively straightforward, but diagnosing the underlying cause can be difficult and often unsuccessful. The long-term effects of hypotonia on a child's development and later life depend primarily on the severity of the muscle weakness and the na ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hyperinsulinism
Hyperinsulinism refers to an above normal level of insulin in the blood of a person or animal. Normal insulin secretion and blood levels are closely related to the level of glucose in the blood, so that a given level of insulin can be normal for one blood glucose level but low or high for another. Hyperinsulinism can be associated with several types of medical problems, which can be roughly divided into two broad and largely non-overlapping categories: those tending toward reduced insulin sensitivity, sensitivity to insulin and high blood glucose levels (hyperglycemia), and those tending toward excessive insulin secretion and low glucose levels (hypoglycemia). Signs and symptoms Hyperinsulinism due to reduced insulin sensitivity is usually asymptomatic. In contrast, hyperinsulinemic hypoglycemia can produce any of the entire range of hypoglycemic symptoms, from shakiness and weakness, to seizures or coma. Diagnosis Diminished sensitivity, associated with diabetes risk Although man ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Reye Syndrome
Reye syndrome is a rapidly worsening brain disease. Symptoms of Reye syndrome may include vomiting, personality changes, confusion, seizures, and loss of consciousness. While liver toxicity typically occurs in the syndrome, jaundice usually does not. Death occurs in 20–40% of those affected with Reye syndrome, and about a third of those who survive are left with a significant degree of brain damage. The cause of Reye syndrome is unknown. It usually begins shortly after recovery from a viral infection, such as influenza or chickenpox. About 90% of cases in children are associated with aspirin (salicylate) use. Inborn errors of metabolism are also a risk factor. The syndrome is associated with changes on blood tests such as a high blood ammonia level, low blood sugar level, and prolonged prothrombin time. Often, the liver is enlarged in the syndrome. Prevention is typically by avoiding the use of aspirin in children. When aspirin was withdrawn for use in children in the U ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
3-hydroxyacyl-coenzyme A Dehydrogenase
In enzymology, a 3-hydroxyacyl-CoA dehydrogenase () is an enzyme that catalyzes the chemical reaction :(S)-3-hydroxyacyl-CoA + NAD+ \rightleftharpoons 3-oxoacyl-CoA + NADH + H+ Thus, the two substrates of this enzyme are (S)-3-hydroxyacyl-CoA and NAD+, whereas its 3 products are 3-oxoacyl-CoA, NADH, and H+. This enzyme belongs to the family of oxidoreductases, to be specific those acting on the CH-OH group of donor with NAD+ or NADP+ as acceptor. Isozymes In humans, the following genes encode proteins with 3-hydroxyacyl-CoA dehydrogenase activity: * HADH – Hydroxyacyl-Coenzyme A dehydrogenase * HSD17B10 – 3-Hydroxyacyl-CoA dehydrogenase type-2 * EHHADH – Peroxisomal bifunctional enzyme * HSD17B4 – Peroxisomal multifunctional enzyme type 2 Function 3-Hydroxyacyl CoA dehydrogenase is classified as an oxidoreductase. It is involved in fatty acid metabolic processes. Specifically it catalyzes the third step of beta oxidation; the oxidation of L-3-hydroxya ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Autosomal Recessive
In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and the second recessive. This state of having two different variants of the same gene on each chromosome is originally caused by a mutation in one of the genes, either new (''de novo'') or inherited. The terms autosomal dominant or autosomal recessive are used to describe gene variants on non-sex chromosomes ( autosomes) and their associated traits, while those on sex chromosomes (allosomes) are termed X-linked dominant, X-linked recessive or Y-linked; these have an inheritance and presentation pattern that depends on the sex of both the parent and the child (see Sex linkage). Since there is only one copy of the Y chromosome, Y-linked traits cannot be dominant or recessive. Additionally, there are other forms of dominance such as incomplete d ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Inborn Error Of Lipid Metabolism
Numerous genetic disorders are caused by errors in fatty acid metabolism. These disorders may be described as fatty oxidation disorders or as a ''lipid storage disorders'', and are any one of several inborn errors of metabolism that result from enzyme defects affecting the ability of the body to oxidize fatty acids in order to produce energy within muscles, liver, and other cell types. Some of the more common fatty acid metabolism disorders are: Coenzyme A dehydrogenase deficiencies * Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD) - Very long-chain acyl-coenzyme A dehydrogenase * Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (LCHAD) - Long-chain 3-hydroxyacyl-coenzyme A * Medium-chain acyl-coenzyme A dehydrogenase deficiency (MCAD) - Medium-chain acyl-coenzyme A dehydrogenase * Short-chain acyl-coenzyme A dehydrogenase deficiency (SCAD) - Short-chain acyl-coenzyme A dehydrogenase * 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (HADH) - 3-hydro ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
