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10630 details
Primary information
ID10630
Therapeutic IDTh1128
Protein NameVelaglucerase alfa
Sequence>Th1128_Velaglucerase_alfa ARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANHTGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGLLSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPEAAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMQYSHSIITNLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFLETISPGYSIHTYLWRRQ
Molecular Weight63000
Chemical FormulaC2532H3850N672O711S16
Isoelectric PointNA
HydrophobicityNA
Melting pointNA
Half-life11-12 Minutes
DescriptionGene-activated human recombinant glucocerebrosidase. It is used to treat Type 1 Gaucher disease, caused by a deficiency of the lysosomal enzyme glucocerebrosidase. Additionally, Velaglucerase alfa has also been investigated for use in Type 3 Gaucher disease.
Indication/DiseaseVelaglucerase alfa is a hydrolytic lysosomal glucocerebroside-specific enzyme indicated for long-term enzyme replacement therapy for pediatric and adult patients with type 1 Gaucher disease.
PharmacodynamicsNA
Mechanism of ActionVelaglucerase alfa catalyzes the hydrolysis of glucocerebroside, reducing the amount of accumulated glucocerebroside.
ToxicityNA
MetabolismNA
AbsorptionNA
The mean volume of distribution at steady state ranges from 82 to 108 mL/kg (8.2% to 10.8% of body weight).
ClearanceMean clearance ranges from 6.72 to 7.56 mL/min/kg.
CategoriesEnzymes
Patents NumberUS7138262
Date of Issue21-11-2006
Date of Expiry18-08-2020
Drug InteractionNA
TargetGlucosylceramidase
Brand NameVPRIV
CompanyShire
Brand DescriptionShire
Prescribed Forlong-term enzyme replacement therapy (ERT) for pediatric and adult patients with type 1 Gaucher disease.
Chemical NameNA
Formulationvelaglucerase alfa (400 Units), citric acid, monohydrate (5.04 mg), polysorbate 20(0.44 mg), sodium citrate, dihydrate (51.76 mg), sucrose (200 mg)
Physical Appearance VPRIV is supplied as a sterile, preservative free, lyophilized powder
Route of AdministrationIntravenous infusion
Recommended DosageThe recommended dose is 60 Units/kg administered every other week as a 60-minute Intravenous infusion.
ContraindicationNA
Side EffectsHeadache, Dizziness, Abdominal pain, Nausea, Back pain, Joint pain (knee), Upper respiratory tract infection, Activated partial thromboplastin time prolonged, Infusion-related reaction*, Pyrexia, Asthenia/Fatigue
Useful Link 1Link
Useful Link 2NA
RemarksNA