フェニル酪酸ナトリウム
WordNet
- a silvery soft waxy metallic element of the alkali metal group; occurs abundantly in natural compounds (especially in salt water); burns with a yellow flame and reacts violently in water; occurs in sea water and in the mineral halite (rock salt) (同)Na, atomic number 11
PrepTutorEJDIC
- ソジウム,ナトリウム(金属元素;化学記号はNa)
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/06/15 21:12:03」(JST)
[Wiki en表示]
Sodium phenylbutyrate
|
Systematic (IUPAC) name |
Sodium 4-phenylbutanoate |
Clinical data |
AHFS/Drugs.com |
Consumer Drug Information |
Licence data |
EMA:Link, US FDA:link |
Pregnancy cat. |
not to be used |
Legal status |
? |
Pharmacokinetic data |
Metabolism |
to phenylacetic acid |
Half-life |
0.8 hours |
Excretion |
80% as phenylacetylglutamine |
Identifiers |
CAS number |
1716-12-7 N |
ATC code |
A16AX03 |
PubChem |
CID 5258 |
ChemSpider |
5068 N |
ChEMBL |
CHEMBL1746 N |
Chemical data |
Formula |
C10H11NaO2 |
Mol. mass |
186.2 g/mol |
SMILES
- [Na+].[O-]C(=O)C(c1ccccc1)CC
|
InChI
-
InChI=1S/C10H12O2.Na/c1-2-9(10(11)12)8-6-4-3-5-7-8;/h3-7,9H,2H2,1H3,(H,11,12);/q;+1/p-1 Y
Key:RHLFTMGPBSLHRS-UHFFFAOYSA-M Y
|
N (what is this?) (verify)
|
Sodium phenylbutyrate is an orphan drug, marketed by Ucyclyd Pharma (Hunt Valley, USA) under the trade name Buphenyl, by Swedish Orphan International (Sweden) as Ammonaps and by Fyrlklövern Scandinavia as triButyrate.
Phenylbutyrate has been used to treat urea cycle disorders.[1]
Contents
- 1 Metabolism
- 2 Research
- 3 References
- 4 See also
|
Metabolism[edit]
Phenylbutyrate is a prodrug. In the human body it is metabolized by beta-oxidation to phenylacetate.
Phenylacetate conjugates with glutamine to phenylacetylglutamine, that is eliminated with the urine.
Research[edit]
Sodium phenylbutyrate is also under investigation for the treatment of some sickle-cell disorders (Blood Products Plasma Expanders and Haemostatics) and for use as a potential differentiation-inducing agent in malignant glioma and acute myeloid leukaemia.[citation needed]
Phenylbutyrate has been associated with longer lifespans in Drosophila.[2]
University of Colorado researchers Dr. Curt Freed and Wenbo Zhou demonstrated that phenylbutyrate stops the progression of Parkinson's disease in mice by turning on a gene called DJ-1 that can protect dopaminergic neurons in the midbrain from dying. As of July 2011[update] they plan on testing phenylbutyrate for the treatment of Parkinson's disease in humans.[3]
References[edit]
- ^ Batshaw, M. L.; MacArthur, R. B.; Tuchman, M. (2001). "Alternative pathway therapy for urea cycle disorders: twenty years later". J. Pediatr. 138 (1 Suppl): S46–S54; discussion S54–S55. doi:10.1067/mpd.2001.111836. PMID 11148549.
- ^ Kang, H. L.; Benzer, S.; Min, K. T. (2002). "Life extension in Drosophila by feeding a drug". Proc. Natl. Acad. Sci. U.S.A. 99 (2): 838–843. doi:10.1073/pnas.022631999. PMC 117392. PMID 11792861.
- ^ News Article
See also[edit]
Glycerol phenylbutyrate
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)
|
|
Various alimentary tract
and metabolism products |
- Anethole trithione
- Glycerol phenylbutyrate
- Miglustat
- Nitisinone
- Sapropterin
- Sodium phenylbutyrate
- Teduglutide
- Tioctic acid
- Zinc acetate
|
|
|
anat (t, g, p)/phys/devp/enzy
|
noco/cong/tumr, sysi/epon
|
proc, drug (A2A/2B/3/4/5/6/7/14/16), blte
|
|
|
|
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- 3,4-dihydroxybenzalacetone and caffeic acid phenethyl ester induce preconditioning ER stress and autophagy in SH-SY5Y cells.
- Tomiyama R1,2, Takakura K1,2, Takatou S1,2, Le TM2, Nishiuchi T3, Nakamura Y4, Konishi T5, Matsugo S1, Hori O2.
- Journal of cellular physiology.J Cell Physiol.2018 Feb;233(2):1671-1684. doi: 10.1002/jcp.26080. Epub 2017 Aug 23.
- PMID 28681934
- First report of carglumic acid in a patient with citrullinemia type 1 (argininosuccinate synthetase deficiency).
- Kose E1, Kuyum P2, Aksoy B2, Häberle J3, Arslan N1, Ozturk Y2.
- Journal of clinical pharmacy and therapeutics.J Clin Pharm Ther.2018 Feb;43(1):124-128. doi: 10.1111/jcpt.12593. Epub 2017 Jul 25.
- PMID 28741715
- 4-Phenybutyric acid promotes gastric cancer cell migration via histone deacetylase inhibition-mediated HER3/HER4 up-regulation.
- Shi X1,2, Zheng C1,2, Li C1,2, Hou K1,2, Wang X1,2, Yang Z1,2, Liu C1,2, Liu Y1,2, Che X1,2, Qu X1,2.
- Cell biology international.Cell Biol Int.2018 Jan;42(1):53-62. doi: 10.1002/cbin.10866. Epub 2017 Oct 9.
- PMID 28851073
Japanese Journal
- オルニチントランスカルバミラーゼ欠損症6症例に対するフェニル酪酸ナトリウムの使用経験
- 小松崎 匠子,大浦 敏博,坂本 修 [他],高柳 正樹,田中 藤樹,奥山 虎之,遠藤 文夫,松原 洋一
- 日本小児科学会雑誌 116(5), 842-848, 2012-05-01
- NAID 10030648516
- Protective Effects of 4-Phenylbutyrate Derivatives on the Neuronal Cell Death and Endoplasmic Reticulum Stress
- Mimori Seisuke,Okuma Yasunobu,Kaneko Masayuki [他],Kawada Koichi,Hosoi Toru,Ozawa Koichiro,Nomura Yasuyuki,Hamana Hiroshi
- Biological and Pharmaceutical Bulletin 35(1), 84-90, 2012
- … Sodium 4-phenylbutyrate (4-PBA) is a terminal aromatic substituted fatty acid that has been used for the treatment of urea cycle disorders. …
- NAID 130001872347
- ヒトα-synuclein(A30P+A53T)Tgマウスの進行性運動機能障害に対する化学シャペロンであるsodium 4-phenylbutyrateの治療効果の検討
Related Links
- View and buy high purity Sodium 4-Phenylbutyrate from Tocris Bioscience, the leading worldwide supplier of high performance life science reagents ... Biological Activity Histone deacetylase inhibitor that displays anticancer activity.
- sodium phenylbutyrate, an agent used as adjunctive treatment to control the hyperammonemia of pediatric urea cycle enzyme disorders. buphenyl An orphan drug for managing urea cycle disorders—e.g., deficiencies of ...
★リンクテーブル★
[★]
フェニルブチレート、フェニル酪酸