Nhận Dạng Quốc Tế & Đặc Tính Hóa Học
Monoisotopic mass
77.029919919
InChI
InChI=1S/C2H7NS/c3-1-2-4/h4H,1-3H2
InChI Key
InChIKey=UFULAYFCSOUIOV-UHFFFAOYSA-N
IUPAC Name
2-aminoethane-1-thiol
Traditional IUPAC Name
cysteamine
Độ hòa tan
Freely soluble in water.
pKa (strongest acidic)
9.42
pKa (Strongest Basic)
10.4
Refractivity
22.39 m3·mol-1
Dược Lực Học :
People born without the ability to metabolize the amino acid cystine suffer from cystinosis, a rare inherited disorder characterized by the deposition and accumulation of cystine crystals throughout the body. These crystals cause considerable damage, particularly in the kidney and eye. Kidney failure can occur by the age of 10 in untreated patients. Cysteamine prevents the accumulation of cystine crystals and is prescribed to prevent further kidney and eye damage. Cysteamine helps to convert cystine into less harmful chemical forms that can be removed from cells.
Cơ Chế Tác Dụng :
Cysteamine is a radiation-protective agent that oxidizes in air to form cystamine. It can be given intravenously or orally to treat radiation sickness. The bitartrate and hydrochloride salt forms are indicated for the treatment of neuropathic cystinosis in patients 6 years old and older. [PubChem]. Cysteamine is marketed under several brand names such as Cystaran™, Procysbi, and Cystagon®.
The free thiol cysteamine depletes cystinotic leukocytes and other cells of cystine, whose accumulation is considered the cause of organ damage in cystinosis. Cysteamine cleaves the disulfide bond with cystine to produce molecules that can escape the metabolic defect in cystinosis and cystinuria.
Dược Động Học :
▧ Absorption :
Cystagon® reaches its maximum plasma concentration in about 1.4 hours.
▧ Volume of Distribution :
Cystagon® has a volume of distribution of 156 L.
▧ Protein binding :
Cysteamine has a plasma protein binding of 52% and is mostly bound to albumin.
▧ Metabolism :
The metabolism of cysteamine has not yet been determined.
▧ Clearance :
The plasma clearance is about 1.2 L/min.
Độc Tính :
Symptoms of overdose may include convulsions (seizures), increased thirst and unusual tiredness or weakness.
Chỉ Định :
Given intravenously or orally to treat radiation sickness. The bitartrate salts (Cystagon® and Procysbi) have been used for the oral treatment of nephropathic cystinosis and cystinurea. The hydrochloride salt (Cystaran™) is indicated for the treatment of corneal cystine crystal accumulation in cystinosis patients.
Liều Lượng & Cách Dùng :
Capsule - Oral - EQ 150MG BASE (CYSTAGON)
Capsule - Oral - EQ 50MG BASE (CYSTAGON )
Capsule, delayed release - Oral - 25MG BASE (PROCYSBI)
Capsule, delayed release - Oral - 75MG BASE (PROCYSBI)
Solution / drops - Ophthalmic - EQ 0.44% BASE (CYSTARAN)
Dữ Kiện Thương Mại
Giá thị trường
-
Giá bán buôn : USD >1.28
Đơn vị tính : capsule
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Giá bán buôn : USD >0.44
Đơn vị tính : capsule
Nhà Sản Xuất
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Sản phẩm biệt dược : Cystagon
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Sản phẩm biệt dược : Cystaran
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Sản phẩm biệt dược : Procysbi
Tài Liệu Tham Khảo Thêm
National Drug Code Directory