2-(2-니트로-4-트리플루오로메틸벤조일)-1,3-시클로헥산디온

기본 정보

• 표준명: 2-(2-니트로-4-트리플루오로메틸벤조일)-1,3-시클로헥산디온
• CAS 레지스트리 번호: 104206-65-7
• 분자식: C14H10F3NO5
• 분자 무게: 329.23
• HS 코드: 2914700090
• Mol 파일: 104206-65-7.mol

동의어: 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione;2-[2-Nitro-4-(trifluoroMethyl)benzoyl]-;1,3-Cyclohexanedione,2-[2-nitro-4-(trifluoroMethyl)benzoyl]-;Nitisinone 2-(2-nitro-4-trifluoroMethylbenzoyl)-1,3-cyclohexanedione;Nitisone;NTBC;SC 0735

관련 CAS 번호: 126534-41-6 1330055-73-6 192190-10-6 101689-95-6 102787-19-9 1120357-23-4 1217536-16-7 13235-07-9 1346597-46-3 12069-69-1

표준명의 화학적 성질

물리적 데이터:

안전정보:

• 픽토그램:
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사용:

• 소개 및 적용: Nitisinone is a medication that acts as a herbicidal triketone and inhibits 4-hydroxyphenylpyruvate dioxygenase (HPPD), an enzyme involved in plastoquinone biosynthesis in plants and tyrosine catabolism in mammals. It is used to treat inherited tyrosinemia type I, an autosomal recessive disease caused by the deficiency of fumarylacetoacetase, the last enzyme in the tyrosine catabolic pathway. Nitisinone prevents the formation of toxic metabolites such as succinylacetoacetate in the liver, and its administration has shown rapid, significant, and persistent inhibition of HPPD in mice. In combination with dietary restriction of tyrosine and phenylalanine, nitisinone treatment has greatly improved the survival of patients and reduced the need for liver transplantation during early childhood. When treatment is started before two years of age, it can reduce the risk of early hepatocellular carcinoma, but there is still a considerable risk of liver malignancy in patients with late start of treatment.