50936-59-9 Usage
Description
Mucopolysaccharidosis II, also known as Hunter Syndrome, is a lysosomal
storage disorder characterized by a deficiency in iduronate-2-sulfatase, an
enzyme responsible for the hydrolysis of the terminal 2-sulfate esters from the
glycosaminoglycans dermatan sulfate and heparin sulfate in the lysosomes of
various cells. This enzyme deficiency causes an accumulation of glycosaminoglycans
(GAGs) in tissue. The clinical manifestations of this deficiency are short
stature, joint stiffness, harsh facial features, hepatosplenomegaly, and progressive
mental retardation. As with other lysosomal storage disorders, the patient’s only
recourse is enzyme replacement therapy (ERT). Idursulfase is a recombinant
human enzyme that has been developed and launched as the ERT for Hunter
syndrome. Unlike most recombinant enzymes, it cannot be produced in prokaryotic
cells. For proper post-translational attachment of N-linked oligosaccharides
and the crucial mannose-6-phosphate groups as the targeting passport into
lysosomes, idursulfase is produced from HT-1080 cells. In addition to being fully
glycosylated with eight mannose-6-phosphate groups, the enzyme possesses
sialylated moieties that improve its stability in circulation.
Originator
Shire
Transkaryotic therapies (UK)
Uses
Treatment of Hunter Syndrome
(enzyme that degrades the glycosaminoglycans heparan
sulfate and dermatan sulfate).
Brand name
Elaprase
Check Digit Verification of cas no
The CAS Registry Mumber 50936-59-9 includes 8 digits separated into 3 groups by hyphens. The first part of the number,starting from the left, has 5 digits, 5,0,9,3 and 6 respectively; the second part has 2 digits, 5 and 9 respectively.
Calculate Digit Verification of CAS Registry Number 50936-59:
(7*5)+(6*0)+(5*9)+(4*3)+(3*6)+(2*5)+(1*9)=129
129 % 10 = 9
So 50936-59-9 is a valid CAS Registry Number.