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Nitisinone

产品号 DB00348 公司名称 DrugBank
CAS号 104206-65-7 公司网站 http://www.ualberta.ca/
分子式 C14H10F3NO5 电 话 (780) 492-3111
分子量 329.2281096 传 真
纯 度 电子邮件 david.wishart@ualberta.ca
保 存 Chembase数据库ID: 232

产品价格信息

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产品别名

标题
Nitisinone
IUPAC标准名
2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione
IUPAC传统名
2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione
商标名
Orfadin
别名
nitisinone

产品登记号

CAS号 104206-65-7
PubChem SID 46507380
PubChem CID 115355

产品性质

疏水性(logP) 1.6

产品详细信息

详细说明 (English)
Item Information
Drug Groups approved; investigational
Description Nitisinone is a synthetic reversible inhibitor of 4-hydroxyphenylpyruvate dioxygenase. It is used in the treatment of hereditary tyrosinemia type 1. It is sold under the brand name Orfadin. [Wikipedia]
Indication Used as an adjunct to dietary restriction of tyrosine and phenylalanine in the treatment of hereditary tyrosinemia type 1.
Pharmacology Hereditary tyrosinemia type 1 occurs due to a deficiency in fumarylacetoacetase (FAH), the final enzyme in the tyrosine catabolic pathway. Nitisinone inhibits catabolism of tyrosine by preventing the catabolic intermediates. In patients with HT-1, these catabolic intermediates are converted to the toxic metabolites succinylacetone and succinylacetoacetate, which are responsible for the observed liver and kidney toxicity. Succinylacetone can also inhibit the porphyrin synthesis pathway leading to the accumulation of 5-aminolevulinate, a neurotoxin responsible for the porphyric crises characteristic of HT-1.
Toxicity Side effects include elevated plasma levels of this amino acid, hepatic and liver failure.
Affected Organisms
Humans and other mammals
Absorption The capsule and liquid formulations are bioequivalent in both the plasma concentration-time curve and maximum plasma concentration (Cmax).
Half Life ~54 hours
External Links
Wikipedia
RxList
Drugs.com

参考文献