Tìm theo
Nitisinone
Các tên gọi khác (4 ) :
  • 2-(alpha,alpha,alpha-Trifluoro-2-nitro-P-tuluoyl)-1,3-cyclohexanedione
  • Nitisinona
  • Nitisinonum
  • Orfadin
Thuốc Gốc
Small Molecule
CAS: 104206-65-7
ATC: A16AX04
ĐG : Apoteket Produktion and Laboratorier Ab , http://www.apl.se
CTHH: C14H10F3NO5
PTK: 329.2281
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]
Nhận Dạng Quốc Tế & Đặc Tính Hóa Học
Công thức hóa học
C14H10F3NO5
Phân tử khối
329.2281
Monoisotopic mass
329.05110705
InChI
InChI=1S/C14H10F3NO5/c15-14(16,17)7-4-5-8(9(6-7)18(22)23)13(21)12-10(19)2-1-3-11(12)20/h4-6,12H,1-3H2
InChI Key
InChIKey=OUBCNLGXQFSTLU-UHFFFAOYSA-N
IUPAC Name
2-{[2-nitro-4-(trifluoromethyl)phenyl]carbonyl}cyclohexane-1,3-dione
Traditional IUPAC Name
nitisinone
SMILES
[O-][N+](=O)C1=C(C=CC(=C1)C(F)(F)F)C(=O)C1C(=O)CCCC1=O
Độ hòa tan
8.11e-03 g/l
logP
1.6
logS
-4.6
pKa (strongest acidic)
2.71
pKa (Strongest Basic)
-7.3
PSA
97.03 Å2
Refractivity
72.35 m3·mol-1
Polarizability
26.74 Å3
Rotatable Bond Count
4
H Bond Acceptor Count
5
H Bond Donor Count
0
Physiological Charge
-1
Number of Rings
2
Bioavailability
1
Rule of Five
true
Ghose Filter
true
Dược Lực Học : 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.
Cơ Chế Tác Dụng : 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] Nitisinone is a competitive inhibitor of 4-hydroxyphenyl-pyruvate dioxygenase, an enzyme upstream of fumarylacetoacetate hydrolyase (FAH) in the tyrosine catabolic pathway. By inhibiting the normal catabolism of tyrosine in patients with hereditary tyrosinemia type 1 (HT-1), nitisinone prevents the accumulation of the catabolic intermediates maleylacetoacetate and fumarylacetoacetate.
Dược Động Học :
▧ Absorption :
The capsule and liquid formulations are bioequivalent in both the plasma concentration-time curve and maximum plasma concentration (Cmax).
▧ Half Life :
~54 hours
Độc Tính : Side effects include elevated plasma levels of this amino acid, hepatic and liver failure.
Chỉ Định : Used as an adjunct to dietary restriction of tyrosine and phenylalanine in the treatment of hereditary tyrosinemia type 1.
Liều Lượng & Cách Dùng : Capsule - Oral
Dữ Kiện Thương Mại
Giá thị trường
  • Biệt dược thương mại : Orfadin 2 mg capsule
    Giá bán buôn : USD >61.74
    Đơn vị tính : capsule
  • Biệt dược thương mại : Orfadin 5 mg capsule
    Giá bán buôn : USD >154.34
    Đơn vị tính : capsule
  • Biệt dược thương mại : Orfadin 10 mg capsule
    Giá bán buôn : USD >308.68
    Đơn vị tính : capsule
Nhà Sản Xuất
  • Công ty :
    Sản phẩm biệt dược : Orfadin
Đóng gói
Tài Liệu Tham Khảo Thêm
drugbank
http://www.drugbank.ca/drugs/DB00348
PubChem Compound
http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi?sid=115355
PubChem Substance
http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi?sid=46507380
ChemSpider
http://www.chemspider.com/Chemical-Structure.103195.html
National Drug Code Directory
http://www.hipaaspace.com/Medical_Billing/Coding/National.Drug.Codes/PDF/66621-1002-6
PharmGKB
http://www.pharmgkb.org/drug/PA164777037
Wikipedia
http://en.wikipedia.org/wiki/Nitisinone
RxList
http://www.rxlist.com/cgi/generic/orfadin.htm
Drugs.com
http://www.drugs.com/cdi/nitisinone.html
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