カルグルミン酸
WordNet
- street name for lysergic acid diethylamide (同)back breaker, battery-acid, dose, dot, Elvis, loony toons, Lucy in the sky with diamonds, pane, superman, window pane, Zen
- any of various water-soluble compounds having a sour taste and capable of turning litmus red and reacting with a base to form a salt
- having the characteristics of an acid; "an acid reaction"
PrepTutorEJDIC
- 酸性の / 酸味のある,すっぱい(sour) / (言葉・態度などが)厳しい,しんらつな / 酸 / すっぱいもの / 《俗》=LSD
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/06/30 03:46:15」(JST)
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Carglumic acid
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| Systematic (IUPAC) name |
|
(2S)-2-(carbamoylamino)pentanedioic acid
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| Clinical data |
| AHFS/Drugs.com |
Consumer Drug Information |
| License data |
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Pregnancy
category |
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Routes of
administration |
Oral |
| Pharmacokinetic data |
| Bioavailability |
30% |
| Protein binding |
Undetermined |
| Metabolism |
Partial |
| Biological half-life |
4.3 to 9.5 hours |
| Excretion |
Fecal (60%) and renal (9%, unchanged) |
| Identifiers |
| CAS Number |
1188-38-1 N |
| ATC code |
A16AA05 (WHO) |
| PubChem |
CID 121396 |
| IUPHAR/BPS |
7458 |
| DrugBank |
DB06775 Y |
| ChemSpider |
1265942 N
108351 |
| UNII |
5L0HB4V1EW N |
| KEGG |
D07130 Y |
| ChEBI |
CHEBI:71028 N |
| ChEMBL |
CHEMBL1201780 N |
| Synonyms |
(S)-2-ureidopentanedioic acid |
| Chemical data |
| Formula |
C6H10N2O5 |
| Molar mass |
190.2 g/mol |
SMILES
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C(CC(=O)O)[C@@H](C(=O)O)NC(=O)N
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InChI
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InChI=1S/C6H10N2O5/c7-6(13)8-3(5(11)12)1-2-4(9)10/h3H,1-2H2,(H,9,10)(H,11,12)(H3,7,8,13)/t3-/m0/s1 Y
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Key:LCQLHJZYVOQKHU-VKHMYHEASA-N Y
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| NY (what is this?) (verify) |
Carglumic acid is an orphan drug, marketed by Orphan Europe under the trade name Carbaglu. Carglumic acid is used for the treatment of hyperammonaemia in patients with N-acetylglutamate synthase deficiency.[1][2] The initial daily dose ranges from 100 to 250 mg/kg, adjusted thereafter to maintain normal plasma levels of ammonia.
The US FDA approved it for treatment of hyperammonaemia on March 18, 2010. Orphan Drug exclusivity expires on March 18, 2017.[3]
References
- ^ Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M (2004). "Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate". J Pediatr 145 (4): 552–4. doi:10.1016/j.jpeds.2004.06.047. PMID 15480384.
- ^ Elpeleg O, Shaag A, Ben-Shalom E, Schmid T, Bachmann C (2002). "N-acetylglutamate synthase deficiency and the treatment of hyperammonemic encephalopathy". Ann Neurol 52 (6): 845–9. doi:10.1002/ana.10406. PMID 12447942.
- ^ "Patent and Exclusivity Search Results".
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Other alimentary tract and metabolism products (A16)
|
|
| Amino acids and derivatives |
- Levocarnitine
- Ademetionine
- Levoglutamide
- Cysteamine
- Carglumic acid
- Betaine
|
|
| Enzymes |
- Carbohydrate metabolism: sucrase (Sacrosidase)
- alpha-glucosidase (Alglucosidase alfa)
- Glycolipid/sphingolipid: glucocerebrosidase (Alglucerase
- Imiglucerase
- Taliglucerase alfa
- Velaglucerase alfa)
- alpha-galactosidase (Agalsidase alfa
- Agalsidase beta)
- Glycosaminoglycan: iduronidase (Laronidase)
- arylsulfatase B (Galsulfase)
- iduronate-2-sulfatase (Idursulfase)
- Lipid: lysosomal lipase (Sebelipase alfa)
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| Other |
- Anethole trithione
- Eliglustat
- Glycerol phenylbutyrate
- Miglustat
- Nitisinone
- Sapropterin
- Sodium benzoate
- Sodium phenylbutyrate
- Teduglutide
- Tioctic acid
- Zinc acetate
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|
UpToDate Contents
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English Journal
- Use of carglumic acid in the treatment of hyperammonaemia during metabolic decompensation of patients with propionic acidaemia.
- Abacan M, Boneh A.SourceMurdoch Childrens Research Institute, Royal Children's Hospital, Melbourne, Australia.
- Molecular genetics and metabolism.Mol Genet Metab.2013 Aug;109(4):397-401. doi: 10.1016/j.ymgme.2013.05.018. Epub 2013 Jun 6.
- Propionic acidaemia (PA) results from propionyl-CoA carboxylase deficiency. During metabolic decompensation, the accumulation of propionyl-CoA causes secondary hyperammonaemia through N-acetylglutamate synthetase inactivation. Carglumic acid, a structural analogue of N-acetylglutamate, was given to
- PMID 23791308
- Recurrent encephalopathy: NAGS (N-acetylglutamate synthase) deficiency in adults.
- Cartagena A, Prasad AN, Rupar CA, Strong M, Tuchman M, Ah Mew N, Prasad C.SourceDepartment of Clinical Neurological Sciences, University of Western Ontario, London. Ontario, Canada.
- The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques.Can J Neurol Sci.2013 Jan;40(1):3-9.
- N-acetyl-glutamate synthase (NAGS) deficiency is a rare autosomal recessive urea cycle disorder (UCD) that uncommonly presents in adulthood. Adult presentations of UCDs include; confusional episodes, neuropsychiatric symptoms and encephalopathy. To date, there have been no detailed neurological desc
- PMID 23250120
- [Carglumic acid for treatment of valproic acid-induced hyperammonaemia in a paediatric patient].
- Gramage Caro T, Vélez-Díaz-Pallarés M, Serna Pérez J, Bermejo Vicedo T.
- Farmacia hospitalaria : órgano oficial de expresión científica de la Sociedad Española de Farmacia Hospitalaria.Farm Hosp.2012 Sep-Oct;36(5):437-8. doi: 10.1016/j.farma.2011.08.005. Epub 2012 Aug 1.
- PMID 22858088
Related Links
- Carglumic acid is a man-made form of an enzyme that occurs naturally in the liver. This enzyme is necessary for processing excess nitrogen produced when the body metabolizes proteins. Without this enzyme, nitrogen builds up in ...
- Read more about the pharmacogenomics of carglumic acid on PharmGKB. ... Disclaimer: The PharmGKB's clinical annotations reflect expert consensus based on clinical evidence and peer-reviewed literature available at the time ...