Oxidase deficiencyTyrosineCompoundsAlcaptonuriaAciduraEnzymeUrineCertain amino acidsPhenylalanineAmino acidWater-solubleAlkaptonuriaAspartic AcidAscorbic acidFattyLysergicDerivativesOrganicToxicProteinTransaminationAlpha hydrCorrosiveEnzymesInflammationHydrogenCarbonProteinsLeadsSourFormImportantComplexSimilarFoundSkinFormationSaltSolutionPlants
Oxidase deficiency1
- Untangling the Spirals of Metabolic Disease: Primary Diagnoses and Secondary Effects: Implications for Treatment David A. H. Whiteman MD 1909 Archibald Garrod In his paper, Inborn Errors of Metabolism, the disease Alkaptonuria (Ochronosis: Homogentisic Acid Oxidase Deficiency) is described as being caused by a gene. (abcdocz.com)
Tyrosine12
- Specifically, 4-hydroxyphenylpyruvate dioxygenase converts a tyrosine byproduct called 4-hydroxyphenylpyruvate to homogentisic acid. (wikipedia.org)
- These diseases occur when the body cannot fully break down certain amino acids including tyrosine. (europa.eu)
- Patients with HT-1 and AKU lack enzymes to break down the amino acid tyrosine properly and, as a result, it is converted to harmful substances. (europa.eu)
- Diet restrictions prevent excessive levels of amino acids such as tyrosine and of phenylalanine, which is converted into tyrosine in the body. (europa.eu)
- Orfadin was effective at reducing the harmful breakdown product of tyrosine called homogentisic acid (HGA). (europa.eu)
- Tyrosine is an amino acid that is naturally produced in the body from another amino acid called phenylalanine. (pinnacleclinic.com)
- Tyrosinemia is a genetic disorder characterized by disruptions in the multistep process that breaks down the amino acid tyrosine, a building block of most proteins. (pinnacleclinic.com)
- the amino acids phenylalanine and tyrosine, which are important building blocks of proteins. (medlineplus.gov)
- As a result, a substance called homogentisic acid, which is produced as phenylalanine and tyrosine are broken down, accumulates in the body. (medlineplus.gov)
- The mutation causes defects in the homogentisate 1,2-dioxygenase enzyme which hampers the breakdown of tyrosine. (hxbenefit.com)
- This enzyme plays an important role in breaking down the important protein building blocks tyrosine and the amino acids phenylalanine. (hxbenefit.com)
- Consequently, the homogentisic acid that is produced as tyrosine and phenylalanine (that are broken down) builds up inside the body. (hxbenefit.com)
Compounds5
- Kernicterus-a rare condition of high levels of bilirubin (compounds resulting from the breakdown of blood color) in the blood. (cheminees-bretaud.com)
- Amino acids are organic compounds that contain amine and carboxyl functional groups, along with a side chain specific to each amino acid. (pinnacleclinic.com)
- As the substrate for other amino acids such as cysteine and taurine, versatile compounds such as SAM-e, and the important antioxidant glutathione, methionine plays a critical role in the metabolism and health of many species, including humans. (pinnacleclinic.com)
- The excessive homogentisic acid and other similar compounds accumulate in the connective tissues, leading to darkening of the cartilage and skin. (hxbenefit.com)
- Succinylacetone, also known as 4,6-dioxoheptanoic acid or SUAC, belongs to the class of compounds known as medium-chain keto acids and derivatives. (hmdb.ca)
Alcaptonuria1
- Homogentisic acid is completely absent from both urine and blood plasma if the individual does not have Alcaptonuria. (hxbenefit.com)
Acidura1
- Family history is the only known risk factor for Homogentisic Acidura. (hxbenefit.com)
Enzyme2
- AKU is caused by the lack of an enzyme called homogentisic acid dioxygenase. (nihr.ac.uk)
- Of the following, the MOST appropriate laboratory study to obtain is A. B. C. D. E. leukocyte lysosomal enzyme activities plasma long-chain fatty acid levels serum carnitine level urine for reducing substances urine organic acid levels A 3-month-old infant who has a history of gastroesophageal reflux has had increasing vomiting for 2 days. (abcdocz.com)
Urine6
- Homogentisic acid is also excreted in urine, making the urine turn dark when exposed to air. (medlineplus.gov)
- Due to these factors, the homogentisic acid, along with its oxide named alkapton, starts accumulating in the blood of patients and are passed in considerable amounts with urine. (hxbenefit.com)
- The acid is also passed along with urine which makes the urine turn dark once exposed to air. (hxbenefit.com)
- Urinalysis and blood tests are performed to detect any sign of homogentisic acid in the patient's urine. (hxbenefit.com)
- C 4 H 8 O 3 , any of the acids present in the urine, esp. (unboundmedicine.com)
- Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. (hmdb.ca)
Certain amino acids1
- The key elements of an amino acid are carbon, hydrogen, oxygen, and nitrogen, although other elements are found in the side chains of certain amino acids. (pinnacleclinic.com)
Phenylalanine1
- Phenylalanine is an amino acid found in many foods and used by your body to produce proteins and other important molecules. (pinnacleclinic.com)
Amino acid8
- Nitisinone is not licensed for the treatment of AKU, but it is licensed for use in babies to treat tyrosinaemia type 1 (HT-1), an amino acid disorder which can cause serious liver damage if left untreated. (nihr.ac.uk)
- is an ɑ-amino acid that is used in the biosynthesis of proteins. (pinnacleclinic.com)
- It can be synthesized in the human body under normal physiological circumstances, making it a nonessential amino acid. (pinnacleclinic.com)
- Aspartic acid, is an α-amino acid that is used in the biosynthesis of proteins. (pinnacleclinic.com)
- Asparagine (Asn) is a central intermediate in amino acid metabolism and an important form of stored or transported nitrogen (N) in higher plants. (pinnacleclinic.com)
- Methionine is an essential amino acid in humans. (pinnacleclinic.com)
- Tryptophan contains an α-amino group, an α-carboxylic acid group, and a side chain indole, making it a non-polar aromatic amino acid. (pinnacleclinic.com)
- C 4 H 7 NO 4 , a nonessential amino acid. (unboundmedicine.com)
Water-soluble1
- ABBR: AHA Any of a class of water-soluble acids derived from fruit or milk, having a hydroxyl moiety in the first position in the molecule. (unboundmedicine.com)
Alkaptonuria1
- Alkaptonuria-a disease characterized by high accumulation of homogentisic acid in the body. (cheminees-bretaud.com)
Aspartic Acid1
- C 10 H 18 N 4 O 6 , a compound intermediate in the synthesis of arginine, formed from citrulline and aspartic acid. (unboundmedicine.com)
Ascorbic acid1
- Hyaluronic acid and ascorbic acid are two examples. (28cubed.com)
Fatty4
- Organic acid disorders Fatty acid oxidation disorders MMBID 8th ed. 2001, chapter 100, p 2289 Zschocke/Hoffmann: Vademecum Metabolicum. (abcdocz.com)
- C 18 H 30 O 2 , an omega-3 fatty acid derived from plants, esp. (unboundmedicine.com)
- C 20 H 32 O 2 , an omega-6 fatty acid formed by the action of enzymes on phospholipids in cell membranes. (unboundmedicine.com)
- in diabetic ketoacidosis, when the conversion of fatty acids to ketones increases. (unboundmedicine.com)
Lysergic1
- 4. Slang term for lysergic acid diethylamide (LSD). (unboundmedicine.com)
Derivatives1
- α-Ketoglutaric acid is one of two ketone derivatives of glutaric acid. (pinnacleclinic.com)
Organic1
- Succinylacetone is a keto-acid, which is a subclass of organic acids. (hmdb.ca)
Toxic2
- This leads to a condition in which patients cannot fully break down a toxic acid called homogentisic acid (HGA). (nihr.ac.uk)
- Boric acid is toxic and should be used only rarely. (unboundmedicine.com)
Protein1
- Reduced nitrogen enters the human body as dietary free amino acids, protein, and the ammonia produced by intestinal tract bacteria. (medmuv.com)
Transamination1
- Transamination, a chemical reaction that transfers an amino group to a ketoacid to form new amino acids. (pinnacleclinic.com)
Alpha hydr1
- a) Light chemical peels that use a moderate solution such as alpha hydroxy acid, glycolic acid, or fruit enzymes. (28cubed.com)
Corrosive1
- C 3 H 4 O 2 , a colorless corrosive acid used in making acrylic polymers and resins. (unboundmedicine.com)
Enzymes1
- A pair of principal enzymes, glutamate dehydrogenase and glutamine synthatase, are found in all organisms and effect the conversion of ammonia into the amino acids glutamate and glutamine, respectively. (medmuv.com)
Inflammation1
- They contain high levels of lycopene, phenolic acid, and homogentisic acid, all of which fight inflammation and protect DNA. (abstracthiphop.com)
Hydrogen1
- An acid containing hydrogen and one other element. (unboundmedicine.com)
Carbon1
- These are keto acids with 6 to 12 carbon atoms. (hmdb.ca)
Proteins1
- It is a building block of proteins, participates in the citric acid and urea cycles, and is a neurotransmitter. (unboundmedicine.com)
Leads1
- The homogentisic acid buildup in tissues and cartilage leads to several serious symptoms and health problems. (hxbenefit.com)
Sour1
- C 2 H 4 O 2 , the acid that gives the sour taste to vinegar. (unboundmedicine.com)
Form2
- An acid reacts with a metal to form a salt, neutralizes bases, and turns litmus paper red. (unboundmedicine.com)
- Nitrogen fixation is carried out by bacterial nitrogenases forming reduced nitrogen, NH4+ which can then be used by all organisms to form amino acids. (medmuv.com)
Important2
- Because black truffle salt contains tannic acid, it's important to store it in an airtight container. (abstracthiphop.com)
- It is important to note that not all of these products contain sulfuric " aria-label="Link to acid "> acid . (synonym.com)
Complex2
- The pathways of different amino acids and other metabolites interweave and overlap to creating complex systems. (pinnacleclinic.com)
- Any of the complex acids that occur as salts in bile, e.g., cholic, glycocholic, and taurocholic acids. (unboundmedicine.com)
Similar1
- Similar to all other amino acids it contains an amino group and a carboxylic acid. (pinnacleclinic.com)
Found2
- Tannic acid, found in black truffle salt, is an antioxidant. (abstracthiphop.com)
- The acid is found in many foods. (unboundmedicine.com)
Skin2
- They also decrease or reduce discolouration, age spots, or blotchiness by exfoliating the top layer of skin as well as the dark spots with glycolic acid and salicylic acid, which speeds up the skin lightening process. (28cubed.com)
- But if you are still on the fence about whether you should join "team Hydroquinone" here is a breakdown of how using it can positively impact your skin. (glam.com)
Formation1
- Corticosteroids inhibit formation of arachidonic acid from phospholipids when cell membranes are damaged. (unboundmedicine.com)
Salt1
- The unique tannic acid in this salt adds to its health benefits, as well as its ability to relieve stress. (abstracthiphop.com)
Solution1
- H 3 BO 3 , a white crystalline acid that in water forms a very weak acid solution poisonous to plants and animals. (unboundmedicine.com)
Plants1
- C 17 H 11 NO 7 , an acid derived from Aristolochia , a genus of flowering plants, and used as an herbal remedy. (unboundmedicine.com)