Acid isovaleric acid. Medical search. Frequent questions

Isovaleric acid is found to be associated with isovaleric acidemia, which is an inborn error of metabolism. (hmdb.ca)
Isovaleric acid is a major component of the cause of intense foot odor, as it is produced by skin bacteria metabolizing leucine and in rare cases a condition called isovaleric acidemia can lead to heightened levels of this metabolite. (wikipedia.org)
What is Isovaleric Acidemia? (news-medical.net)
Tanaka and colleagues first described isovaleric acidemia (IVA), a rare genetic condition of leucine metabolism, in 1966. (news-medical.net)
Isovaleric acidemia can cause various health concerns, ranging from minor to life-threatening. (news-medical.net)
Isovaleric acidemia is caused by IVD (isovaleryl-CoA dehydrogenase) gene mutations. (news-medical.net)
Isovaleric acidemia has an autosomal recessive inheritance pattern. (news-medical.net)
In other situations, the signs and symptoms of isovaleric acidemia first present in childhood and then fade away. (news-medical.net)
Isovaleric acidemia is estimated to have a prevalence of around one in 100,000 persons. (news-medical.net)
The diagnosis of isovaleric acidemia was made based on the patient's medical history, physical examination, and test results. (news-medical.net)
The characteristic symptoms, clinical exam, and blood and urine testing are used to diagnose isovaleric acidemia. (news-medical.net)
Deficiency of this dehydrogenase results in isovaleric acidemia, also known as "sweaty feet" syndrome, because accumulated isovaleric acid emits an odor that smells like sweat. (msdmanuals.com)
In isovaleric acidemia, a lack of isovaleryl-CoA dehydrogenase, which is involved in the metabolism of the branched chain amino acid leucine, results in the inability to break down isovaleric acid and the accumulation of potentially toxic metabolites. (medicalhomeportal.org)
With early diagnosis and ongoing treatment of isovaleric acidemia, most affected children will have normal development. (medicalhomeportal.org)
To confirm the diagnosis of isovaleric acidemia, work with Newborn Screening Services ( see NV providers [2] ) . (medicalhomeportal.org)
See Isovaleric Acidemia - Information for Parents (STAR-G) for additional information. (medicalhomeportal.org)
They did tell us that they thought he had Isovaleric Acidemia and gave us some paperwork explaining the condition. (cincinnatichildrens.org)
Their oldest son Micah has Isovaleric Acidemia. (cincinnatichildrens.org)

0.05) influence on all of the VFAs produced excluding butyric acid. (helsinki.fi)
The global market for carboxylic acid is chiefly driven by the increased consumption of vinyl acetate monomer (VAM) in the food and beverages industry and the rising demand for valeric, butyric, and stearic acid in cosmetics and personal care products. (openpr.com)
Colorless Transparent Liquid Butyric Acid Manuf. (interchinachem.com)

Certain varieties of carboxylic acids such as benzoic acid, acetic acid, and citric acid are also used as preservatives in the food and beverages industry. (openpr.com)
Some carboxylic acids such as acetic acid, benzoic acid, and citric acid find wide usage as preservatives in the food and beverages industry. (openpr.com)

Isovaleric acid is slightly soluble in water. (guidechem.com)
Isovaleric acid is clear colorless liquid that is sparingly soluble in water, but well soluble in most common organic solvents. (hmdb.ca)
This is important in brewing as a-acids are not very soluble in water whereas iso-a-acids are very soluble. (castlemalting.com)

Isovaleric acidaemia (IVA), is one of a number of disorders where, due to the lack of an enzyme, the body cannot properly deal with one of the amino acids, leucine. (nestlehealthscience.com)

Isovaleric acid has a strong pungent cheesy or sweaty smell, but its volatile esters such as ethyl isovalerate have pleasant odors and are widely used in perfumery. (wikipedia.org)
Since isovaleric acid and its esters are natural components of many foods, it is present in mammals including humans. (wikipedia.org)

Short chain fatty acids (SCFAs) are produced in the colon by the gut microbiota. (metabolon.com)
On days 8 and 16 of life the morphometry, intestinal architecture, and cytokine release, mesenteric lymph nodes cell composition, plasma immunoglobulin concentrations, fecal microbiota composition, cecal short-chain fatty acids content, and the urinary metabolic profile were assessed. (frontiersin.org)
The objective of the present study was to investigate the effects of inulin and isomalto-oligosaccharide on gastrointestinal motility-related hormones, short-chain fatty acids, fecal microflora, and reproductive performance in pregnant sows. (asas.org)

Certified wine flavour standard used to train professional wine tasters to recognize and scale the intensity of isovaleric character. (aroxa.com)
The Isovaleric acid flavour standard helps train professional tasters to recognise the potency of the isovaleric acid character. (flavoractiv.com)

One group of these disorders is amino acid metabolism disorders. (medlineplus.gov)
If you have one of these disorders, your body may have trouble breaking down certain amino acids. (medlineplus.gov)
Protein substitutes for IVA and related disorders, provide all the amino acids found in protein but no leucine. (nestlehealthscience.com)
Overview of Amino Acid and Organic Acid Metabolism Disorders The kidneys actively reabsorb significant amounts of amino acids. (msdmanuals.com)
disorders caused by deficiency of one or more subunits of a dehydrogenase active in the 2nd step of branched-chain amino acid catabolism. (msdmanuals.com)
In secondary carnitine deficiency, which is caused by other metabolic disorders (eg, fatty acid oxidation disorders, organic acidemias), carnitine depletion may be secondary to the formation of acylcarnitine adducts and the inhibition of carnitine transport in renal cells by acylcarnitines. (medscape.com)
In disorders of fatty acid oxidation, excessive lipid accumulation occurs in muscle, heart, and liver, with cardiac and skeletal myopathy and hepatomegaly. (medscape.com)
ABSTRACT The national neonatal screening programme in the United Arab Emirates currently includes 16 disorders: congenital hypothyroidism, sickle-cell diseases, congenital adrenal hyperplasia, biotinidase deficiency and 12 amino acid, organic acid and fatty acid disorders. (who.int)
The incidence of screened disorders were 1:1 873 for congenital hypothyroidism, 1:14 544 for phenylketonuria, 1:3 526 for amino acid, organic acid and fatty acid disorders, 1:9 030 for classical congenital adrenal hyperplasia, 1:8 300 for biotinidase deficiency, 1:2 384 for sickle-cell disease and 1:121 for sickle-cell traits. (who.int)

Isovaleric acid has a characteristic disagreeable odor. (guidechem.com)
Isovaleric acid has a characteristic disagreeable, rancid, cheese-like odor. (guidechem.com)
Isovaleric acid is a colorless liquid with a penetrating odor. (guidechem.com)
Like other low-molecular-weight carboxylic acids, it has an unpleasant odor. (wikipedia.org)
3-Methylbutanoic acid is a minor constituent of the perennial flowering plant valerian (Valeriana officinalis), from which it got its trivial name isovaleric acid: an isomer of valeric acid which shares its unpleasant odor. (wikipedia.org)
In 2006, foot odor without pitted skin changes was discovered to be from overgrowth of Bacillus subtilis and specifically an isovaleric acid produced by Staphylococcus epidermidis overgrowth, a normal skin flora. (medscape.com)
Based on test method ISO17299-3A using isovaleric acid, treated synthetic fibers more than double the odor control efficiency versus current industry standards. (asianetnews.net)

Macleaya cordata extract or benzoic acid have anti-inflammatory, antioxidant, and antimicrobial activities that makes them potential antibiotic alternatives. (frontiersin.org)
The objective of this study was to evaluate the potential effects of feed supplemented with Macleaya cordata extract and benzoic acid on growth performance, immunity, antioxidant capacity, intestinal morphology, and microflora in weaned piglets. (frontiersin.org)
Twenty-four weaned piglets [Duroc × (Large White × Landrace)] 28 days of age and weighing 8.41 ± 0.13 kg were randomly divided in equal numbers ( n = 8) into three groups fed a basal diet (CON), CON + 20 mg/kg flavomycin + 50 mg/kg quinocetone (AGP), or CON + 50 mg/kg Macleaya cordata extract + 1,000 mg/kg benzoic acid (MB). (frontiersin.org)

At low levels isovaleric acid can contribute to wine complexity but at higher concentrations it imparts an odour of stale cheese or sweaty socks. (aroxa.com)
Isovaleric acid Imparts an odour of stale cheese or sweaty socks. (aroxa.com)

Colorless Transparent Liquid Iso-valeric Acid S. (interchinachem.com)

1] Intracellular carnitine deficiency impairs the entry of long-chain fatty acids into the mitochondrial matrix. (medscape.com)
Valproic acid may cause an acquired type of secondary carnitine deficiency by directly impairing renal tubular reabsorption of carnitine. (medscape.com)

It is essential for fat metabolism, serving as a transport agent to carry long-chain fatty acids into the mitochondria, a key step in oxidative metabolism. (mindfreedom.org)
It plays an essential role in the transfer of long-chain fatty acids into the mitochondria for beta-oxidation. (medscape.com)
Consequently, long-chain fatty acids are not available for beta-oxidation and energy production, and the production of ketone bodies (which are used by the brain) is also impaired. (medscape.com)

Ethyl isovalerate Menthyl isovalerate Isovaleryl-CoA Testosterone isovalerate 2-Methylbutanoic acid, an isomer Valeric acid, an isomer Sigma-Aldrich. (wikipedia.org)
Place 100 g. (108 ml.) of dry iso-valeric acid and 12 g of purified red phosphorus in the flask. (thevespiary.org)

3-Methylbutanoic acid, also known as β-methylbutyric acid or more commonly isovaleric acid, is a branched-chain alkyl carboxylic acid with the chemical formula (CH3)2CHCH2CO2H. (wikipedia.org)
CH3)2C=CH2 + H2 + CO → (CH3)2CHCH2CHO → 3-methylbutanoic acid 3-Methylbutanoic acid reacts as a typical carboxylic acid: it can form amide, ester, anhydride, and chloride derivatives. (wikipedia.org)
The global market for carboxylic acid is characterized by stiff competition on account of the presence of a large number of players who are involved in increasing their production capacities to outgun their rivals. (openpr.com)
Carboxylic acid is also used as an active ingredient in a number of drug formulations such as antibiotics, anticoagulants, cholesterol-lowering statins, and non-steroidal anti-inflammatory drugs (NSAIDs). (openpr.com)
Asia Pacific, powered by China, is a dominant manufacturer of carboxylic acid, at present. (openpr.com)
China not only produces maximum carboxylic acid, but also consumes it because of key end-use industries such as consumer goods, lubricants, food and beverages. (openpr.com)
The rising consumer preference to bio-based products will be the key factor driving the increased use of carboxylic acid in the cosmetics and personal care industries, especially in developed economies. (openpr.com)
This method, as the researchers claim, is unique because it uses enzymes as an environment-friendly method to develop heavy-chain carboxylic acid, a monomer which is considered the basic building block for the manufacture of a variety of high-quality plastics. (openpr.com)

May be synthesized by oxidation of isoamyl alcohol or isovaleric aldehyde. (guidechem.com)
2007) decided to test this by surveying 377 individuals for their ability to detect four odorants: isoamyl acetate, isovaleric acid, L-carvone, and cineole. (blogspot.com)

These phenomena may be prevented by killing any Brettanomyces yeasts, such as by sterile filtration, by the addition of relatively large quantities of sulfur dioxide and sometimes sorbic acid, by mixing in alcoholic spirit to give a fortified wine of sufficient strength to kill all yeast and bacteria, or by pasteurization. (wikipedia.org)
Lactic acid, sulfur dioxide and isovaleric acid are the initial aromatics here with lemon, grassy hops, and a winey Sauvignon Blanc character to follow. (beerconnoisseur.com)

Isovaleric acid [ 3-Methylbutanoic acid ] smells like sweat. (blogspot.com)

Also, Isovaleryl-coenzyme A is an intermediate in the metabolism of branched-chain amino acids. (wikipedia.org)

Or there may be a problem getting the amino acids into your cells. (medlineplus.gov)

The acid has been used to synthesize β-hydroxyisovaleric acid - otherwise known as β-hydroxy β-methylbutyric acid - via microbial oxidation by the fungus Galactomyces reessii. (wikipedia.org)

An isovalerate or 3-methylbutanoate ion is (CH3)2CHCH2COO−, the conjugate base of the acid. (wikipedia.org)
An isovalerate or 3-methylbutanoate compound is a salt or ester of the acid. (wikipedia.org)

In fact, the concentrations of isovaleric acid that can be detected differs by as much as 10,000-fold from one individual to the next. (blogspot.com)
It's quite likely that many people reading this blog can't smell isovaleric acid at low concentrations because they don't have one of the olfactory receptors for that ligand [ A Sense of Smell: Olfactory Receptors ]. (blogspot.com)

A 2-oxo monocarboxylic acid that is the 2-oxo derivative of isovaleric acid. (ebi.ac.uk)
Carnitine is a naturally occurring hydrophilic amino acid derivative, produced endogenously in the kidneys and liver and derived from meat and dairy products in the diet. (medscape.com)

Diagnosis of maple syrup urine disease is by finding elevated plasma levels of branched-chain amino acids (particularly leucine) and confirmed by genetic testing. (msdmanuals.com)

Isovaleric acid is used extensively as a flavoring ingredient innonalcoholic beverages and in foods such as ice cream,candy, baked goods, and cheese, as a fragrance ingredientin perfumes, and as a chemical intermediate in themanufacture of sedatives and other pharmaceutical products.It is also used as an extractant of mercaptans from petroleumhydrocarbons, a vinyl stabilizer, and as an intermediate in themanufacture of plasticizers and synthetic lubricants. (guidechem.com)

Sandwalk: Can You Smell Isovaleric Acid? (blogspot.com)
An excess of isovaleric acid in wine is generally seen as a defect, as it can smell sweaty, leathery, or "like a barnyard", but in small amounts it can smell smokey, spicy, or medicinal. (wikipedia.org)

This enzyme aids in processing the amino acid leucine, which is found in many proteins. (news-medical.net)
eg PKU - without this enzyme an amino acid (called phenylalanine) rises to harmful levels and can lead to developmental delay. (nsu.govt.nz)

Defects of amino acid transport in the renal tubule include cystinuria and Hartnup disease, which are discussed elsewhere. (msdmanuals.com)

To repeat a very important point, though, because it bears repeating: valproic acid causes carnitine depletion, especially in children and particularly in combination with other medications, which is common in medical practice. (mindfreedom.org)

Belongs to the class of organic compounds known as methyl-branched fatty acids. (hmdb.ca)
Organic compounds with acidic properties, and will commonly have carboxylic acids or similar functional groups in their structure. (fishersci.com)
Research activities have always been abundant in the area of developing commonly used organic compounds such as carboxylic acids from raw materials derived from natural sources, replacing the use of possibly hazardous chemicals. (openpr.com)

Some strong hoppy craft beers have a noticeable amount of Isovaleric acid within them, adding a slight cheese flavour note to the beer. (flavoractiv.com)

Diseases3

Chemicals and Drugs8

Analytical, Diagnostic and Therapeutic Techniques and Equipment1

Acidemia18

Butyric3

Acetic Acid2

Soluble in water3

Acidaemia1

Esters2

Short Chain3

Flavour standard2

Disorders9

Odor7

Benzoic acid3

Sweaty socks2

Colorless1

Deficiency2

Long-chain3

Valeric acid2

Carboxylic acid8

Isoamyl2

Sulfur dioxide2

Smells like sweat1

Metabolism1

Getting the amino1

Oxidation1

Isovalerate2

Concentrations2

Derivative2

Diagnosis1

Extensively1

Smell2

Enzyme2

Defects1

Carnitine depletion1

Organic compounds3

Cheese1