A16A Other alimentary tract and metabolism products
A16AA
Amino acid
Amino acids are organic compounds that contain both amino and carboxylic acid functional groups. Although hundreds of amino acids exist in nature, by far the most important are the alpha-amino acids, which comprise proteins. Only 22 alpha ...
s and derivatives
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Levocarnitine
Carnitine is a quaternary ammonium compound involved in metabolism in most mammals, plants, and some bacteria. In support of energy metabolism, carnitine transports long-chain fatty acids into mitochondria to be oxidized for energy production ...
:A16AA02
Ademetionine
''S''-Adenosyl methionine (SAM), also known under the commercial names of SAMe, SAM-e, or AdoMet, is a common cosubstrate involved in methyl group transfers, transsulfuration, and aminopropylation. Although these anabolic reactions occur throu ...
:A16AA03
Levoglutamide
Glutamine (symbol Gln or Q) is an α-amino acid that is used in the biosynthesis of proteins. Its side chain is similar to that of glutamic acid, except the carboxylic acid group is replaced by an amide. It is classified as a charge-neutra ...
:A16AA04
Mercaptamine
Cysteamine is a chemical compound that can be biosynthesized in mammals, including humans, by the degradation of coenzyme A. The intermediate pantetheine is broken down into cysteamine and pantothenic acid. It is the biosynthetic precursor to ...
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Carglumic acid
Carglumic acid, sold under the brand name Carbaglu among others, is used for the treatment of hyperammonaemia.
Carglumic acid is a carbamoyl phosphate synthetase 1 (CPS 1) activator.
The most common adverse effects include vomiting, abdominal pa ...
:A16AA06
Betaine
A betaine () in chemistry is any neutral chemical compound with a positively charged cationic functional group, such as a quaternary ammonium or phosphonium cation (generally: onium ions) that bears no hydrogen atom and with a negatively char ...
:A16AA07
Metreleptin
:QA16AA51 Levocarnitine, combinations
A16AB
Enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrate (chemistry), substrates, and the enzyme converts the substrates into different molecule ...
s
:A16AB01
Alglucerase
Alglucerase was a biopharmaceutical drug for the treatment of Gaucher's disease. It was a modified form of human β-glucocerebrosidase enzyme, where the non-reducing ends of the oligosaccharide chains have been terminated with mannose residues.
...
:A16AB02
Imiglucerase
Imiglucerase is a medication used in the treatment of Gaucher's disease.
It is a recombinant DNA-produced analogue of the human enzyme β-glucocerebrosidase.
Cerezyme is a freeze-dried medicine containing imiglucerase, manufactured by Genzyme Co ...
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Agalsidase alfa
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Agalsidase beta
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Laronidase
Iduronidase (, ''L-iduronidase'', ''alpha-L-iduronidase'', ''laronidase''), sold as Aldurazyme, is an enzyme with the systematic name ''glycosaminoglycan alpha-L-iduronohydrolase''. This enzyme catalyses the hydrolysis of unsulfated alpha-L-idu ...
:A16AB06
Sacrosidase
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Alglucosidase alfa
Alglucosidase alfa, sold under the brand name Myozyme among others, is an enzyme replacement therapy (ERT) orphan drug for treatment of Pompe disease (Glycogen storage disease type II), a rare lysosomal storage disorder (LSD).
Chemically speaki ...
:A16AB08
Galsulfase
Arylsulfatase B (N-acetylgalactosamine-4-sulfatase, chondroitinsulfatase, chondroitinase, acetylgalactosamine 4-sulfatase, N-acetylgalactosamine 4-sulfate sulfohydrolase, ) is an enzyme associated with mucopolysaccharidosis VI (Maroteaux– ...
:A16AB09
Idursulfase
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Velaglucerase alfa
Velaglucerase alfa, sold under the brand name Vpriv and manufactured by Shire plc, is a hydrolytic lysosomal glucocerebroside-specific enzyme, which is a recombinant form of glucocerebrosidase indicated as a long-term enzyme replacement therapy ...
:A16AB11
Taliglucerase alfa
Taliglucerase alfa, sold under the brand name Elelyso among others, is a biopharmaceutical medication developed by Protalix and Pfizer. The drug, a recombinant glucocerebrosidase used to treat Gaucher's disease, is the first plant-made pharmaceut ...
:A16AB12
Elosulfase alfa
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Asfotase alfa
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Sebelipase alfa
Sebelipase alfa, sold under the brand name Kanuma, is a recombinant form of the enzyme lysosomal acid lipase (LAL) that is used as a medication for the treatment of lysosomal acid lipase deficiency (LAL-D). Text was copied from this source ...
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Velmanase alfa
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Idursulfase beta
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Cerliponase alfa
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Vestronidase alfa
:A16AB19
Pegvaliase
Pegvaliase, sold under the brand name Palynziq, is a medication for the treatment of the genetic disease phenylketonuria. Chemically, it is a pegylated derivative of the enzyme phenylalanine ammonia-lyase that metabolizes phenylalanine
Pheny ...
:A16AB20
Pegunigalsidase alfa
:A16AB21
Atidarsagene autotemcel
Atidarsagene autotemcel, sold under the brand name Libmeldy, is a gene therapy treatment for metachromatic leukodystrophy (MLD) developed by Orchard Therapeutics. It contains an autologous CD34⁺ cell enriched population that contains haemato ...
:A16AB22
Avalglucosidase alfa
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Cipaglucosidase alfa
Cipaglucosidase alfa, sold under the brand name Pombiliti, and used in combination with miglustat, is a medication used for the treatment of glycogen storage disease type II (Pompe disease). Cipaglucosidase alfa is a recombinant human acid � ...
:A16AB24
Pegzilarginase
Pegzilarginase, sold under the brand name Loargys, is a medication used for the treatment of hyperargininemia.
The most common side effects include allergic reactions.
Pegzilarginase was approved for medical use in the European Union in De ...
:A16AB25
Olipudase alfa
Olipudase alfa, sold under the brand name Xenpozyme, is a medication used for the treatment of non-central nervous system (CNS) manifestations of acid sphingomyelinase deficiency (ASMD) type A/B or type B. Text was copied from this source which ...
A16AX Various alimentary tract and metabolism products
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Thioctic acid
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Anethole trithione
Anethole trithione, anetholtrithione, or anetholtrithion ( JAN) is a drug used in the treatment of dry mouth. It is listed in the U.S. National Cancer Institute's ''Dictionary of Cancer Terms'' as being studied in the treatment of cancer.
:A16AX03
Sodium phenylbutyrate
Sodium phenylbutyrate, sold under the brand name Buphenyl among others, is a salt of an aromatic fatty acid, 4-phenylbutyrate (4-PBA) or 4-phenylbutyric acid. The compound is used to treat urea cycle disorders, because its metabolites offer an ...
:A16AX04
Nitisinone
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Zinc acetate
Zinc acetate is a salt with the formula Zn(CH3CO2)2, which commonly occurs as the dihydrate Zn(CH3CO2)2·2H2O. Both the hydrate and the anhydrous forms are colorless solids that are used as dietary supplements. When used as a food additive, it h ...
:A16AX06
Miglustat
Miglustat, sold under the brand name Zavesca, is a medication used to treat type I Gaucher disease (GD1). It is also known as N-butyldeoxynojirimycin, and is a derivative of the anti-diabetic 1-deoxynojirimycin. It was developed by Oxford GlycoS ...
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Sapropterin
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Teduglutide
Teduglutide (brand names Gattex in the US and Revestive in Europe) is a 33-membered polypeptide and glucagon-like peptide-2 (GLP-2) analog that is used for the treatment of short bowel syndrome. It works by promoting mucosal growth and possibly r ...
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Glycerol phenylbutyrate
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Eliglustat
Eliglustat, sold under the brand name Cerdelga, is a medication used for the treatment of Gaucher's disease. It was discovered at the University of Michigan, developed by Genzyme Corp, and was approved by the FDA in August 2014. Commonly used as ...
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Sodium benzoate
Sodium benzoate is the sodium salt of benzoic acid, widely used as a food preservative (with an E number of E211) and a pickling agent. It appears as a white crystalline chemical with the formula C6H5COONa.
Production
Sodium benzoate is commo ...
:A16AX12
Trientine
Triethylenetetramine (TETA and trien), also known as trientine (INN) when used medically, is an organic compound with the formula H2NHCH2CH2NH2sub>2. The pure freebase is a colorless oily liquid, but, like many amines, older samples assume a yel ...
:A16AX13
Uridine triacetate
:A16AX14
Migalastat
Migalastat, sold under the brand name Galafold, is a medication for the treatment of Fabry disease, a rare genetic disorder. It was developed by Amicus Therapeutics. The US Food and Drug Administration (FDA) granted it orphan drug status in 2004, ...
:A16AX15
Telotristat
Telotristat ethyl ( USAN, brand name Xermelo) is a prodrug of telotristat, which is an inhibitor of tryptophan hydroxylase. It is formulated as telotristat etiprate — a hippurate salt of telotristat ethyl.
On February 28, 2017, the U.S. Foo ...
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Givosiran
Givosiran, sold under the brand name Givlaari, is a medication used for the treatment of adults with acute hepatic porphyria. Givosiran is a small interfering RNA (siRNA) directed towards delta-aminolevulinate synthase 1 (ALAS1), an important ...
:A16AX17
Triheptanoin
Triheptanoin, sold under the brand name Dojolvi, is a medication for the treatment of children and adults with molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD).
The most common adverse reactions include abdominal pain, d ...
:A16AX18
Lumasiran
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Fosdenopterin
Fosdenopterin (or cyclic pyranopterin monophosphate, cPMP), sold under the brand name Nulibry, is a medication used to reduce the risk of death due to a rare genetic disease known as molybdenum cofactor deficiency type A.
The most common sid ...
:A16AX20
Lonafarnib
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Elivaldogene autotemcel
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Tiomolibdic acid
Tiomolibdic acid (trade name Decuprate) is a chelating agent under investigation for the treatment of cancer and of Wilson's disease, a rare and potentially fatal disease in which the body cannot regulate copper. It is developed by Wilson T ...
:A16AX30
Sodium benzoate and sodium phenylacetate
QA16Q Other alimentary tract and metabolism products for veterinary use
QA16QA Drugs for prevention and/or treatment of acetonemia
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Propylene glycol
Propylene glycol ( IUPAC name: propane-1,2-diol) is a viscous, colorless liquid, which is nearly odorless but possesses a faintly sweet taste. Its chemical formula is CH3CH(OH)CH2OH.
Containing two alcohol groups, it is classed as a diol. It ...
:QA16QA02
Sodium propionate
Sodium propanoate or sodium propionate is the sodium salt of propionic acid which has the chemical formula Na( C2 H5 C O O). This white crystalline solid is deliquescent in moist air.
Reactions
It is produced by the reaction of propionic acid a ...
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Glycerol
Glycerol (), also called glycerine in British English and glycerin in American English, is a simple triol compound. It is a colorless, odorless, viscous liquid that is sweet-tasting and non-toxic. The glycerol backbone is found in lipids know ...
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Ammonium lactate
Ammonium lactate is a Compound (chemistry), compound with formula NH4(C2H4(OH)COO). It is the ammonium salt of lactic acid. It has mild anti-bacterial properties.
It has E number "E328" and is the active ingredient of the skin lotions Amlactin a ...
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Clanobutin
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Monensin
:QA16QA52 Sodium propionate, combinations
References
{{Other alimentary tract and metabolism products
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