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Recombinant Human GBA therapeutic protein(Velaglucerase alfa)

Cat.No. : GBA-P024H
Product Overview : The therapeutic protein is a hydrolytic lysosomal glucocerebroside-specific enzyme, which is a recombinant form of glucocerebrosidase indicated as a long-term enzyme replacement therapy for those suffering of Gaucher disease Type 1. It has an identical amino acid sequence to the naturally occurring enzyme. It is used for the treatment of 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.
Availability February 17, 2025
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Description : This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants.
Species : Human
Molecular Mass : ~63 kDa
AA Sequence : ARPCIPKSFGYSSVVCVCNATYCDS FDPPTFPALGTFSRYESTRSGRRME LSMGPIQANHTGTGLLLTLQP EQKFQKVKGFGGAMTDAAALNILAL SPPAQNLLLKSYFSEEGIGYNIIRV PMASCDFSIRTYTYADTPDDF QLHNFSLPEEDTKLKIPLIHRALQL AQRPVSLLASPWTSPTWLKTNGAVN GKGSLKGQPGDIYHQTWARYF VKFLDAYAEHKLQFWAVTAENEPSA GLLSGYPFQCLGFTPEHQRDFIARD LGPTLANSTHHNVRLLMLDDQ RLLLPHWAKVVLTDPEAAKYVHGIA VHWYLDFLAPAKATLGETHRLFPNT MLFASEACVGSKFWEQSVRLG SWDRGMQYSHSIITNLLYHVVGWTD WNLALNPEGGPNWVRNFVDSPIIVD ITKDTFYKQPMFYHLGH FSKFIPEGSQRVGLVASQKNDLDAV ALMHPDGSAVVVVLNRSSKDVPLTI KDPAVGFLETISPGYSIHTYL WRRQ
Endotoxin : < 0.1 EU per μg of the protein
Purity : >96%
Tag : Non
Alias : GBA; GBA1; GCB; GLUC; Velaglucerase alfa
Gene Name : GBA glucosidase, beta, acid [ Homo sapiens ]
Official Symbol : GBA
Synonyms : GBA; glucosidase, beta, acid; GLUC, glucosidase, beta; acid (includes glucosylceramidase) , glucosylceramidase; glucosylceramidase; GBA1; alglucerase; imiglucerase; acid beta-glucosidase; beta-glucocerebrosidase; lysosomal glucocerebrosidase; D-glucosyl-N-acylsphingosine glucohydrolase; GCB; GLUC;
Gene ID : 2629
mRNA Refseq : NM_000157
Protein Refseq : NP_000148
MIM : 606463
UniProt ID : P04062
Chromosome Location : 1q22
Pathway : Glycosphingolipid metabolism, organism-specific biosystem; Lysosome, organism-specific biosystem; Lysosome, conserved biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem; Metabolism of lipids and lipoproteins, organism-specific biosystem; Other glycan degradation, organism-specific biosystem;
Function : cation binding; glucosylceramidase activity; hydrolase activity, acting on glycosyl bonds; receptor binding;

Not For Human Consumption!

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06/08/2022

    This guidance allows me to design and implement experiments effectively while adhering to best practices, enhancing the reliability and reproducibility of my results.

    08/08/2021

      They ensure the purity, integrity, and functionality of GBA protein through rigorous quality control measures.

      05/02/2018

        The manufacturer's commitment to quality control is essential in achieving reliable and consistent outcomes

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