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Recombinant Human Glucosylceramidase/GBA Protein, CF

Catalog #: 7410-GHB
11 Citations Datasheet / COA / SDS
Catalog # Availability Size / Price Qty
7410-GHB-020
7410-GHB-01M
Recombinant Human Glucosylceramidase/GBA Protein Enzyme Activity Diagram.
3 Images
Product Details
Citations (11)
FAQs
Reviews
Summary Product Datasheets Carrier Free Data Images Reconstitution Calculator Background Related Research Areas

Recombinant Human Glucosylceramidase/GBA Protein, CF Summary

Product Specifications

Accession #
P04062.3
N-terminal Sequence
Analysis
Ala40
Predicted Molecular Mass
56 kDa
SDS-PAGE
60-75 kDa, reducing conditions

Product Datasheets

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7410-GHB

Product Datasheet
COA
SDS

Carrier Free

What does CF mean?

CF stands for Carrier Free (CF). We typically add Bovine Serum Albumin (BSA) as a carrier protein to our recombinant proteins. Adding a carrier protein enhances protein stability, increases shelf-life, and allows the recombinant protein to be stored at a more dilute concentration. The carrier free version does not contain BSA.

What formulation is right for me?

In general, we advise purchasing the recombinant protein with BSA for use in cell or tissue culture, or as an ELISA standard. In contrast, the carrier free protein is recommended for applications, in which the presence of BSA could interfere.

7410-GHB

Formulation Supplied as a 0.2 μm filtered solution in Sodium Citrate.
Shipping The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage: Store the unopened product at -70 °C. Use a manual defrost freezer and avoid repeated freeze-thaw cycles. Do not use past expiration date.

Scientific Data

Enzyme Activity View Larger

Recombinant Human Glucosylceramidase/GBA Protein, CF (Catalog # 7410-GHB) cleaves the beta-glucosidic linkage of glucosylceramide, an intermediate in glycolipid metabolism.

Enzyme Activity View Larger

Recombinant Human Glucosylceramidase/GBA Protein, CF (Catalog # 7410-GHB) is measured by its ability to hydrolyze 4-methylumbelliferyl-beta -D-glucopyranoside.

SDS-PAGE View Larger

2 μg/lane of Recombinant Human Glucosylceramidase/GBA Protein (Catalog # 7410-GHB) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 60-75 kDa.

Reconstitution Calculator

Reconstitution Calculator

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

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Background: Glucosylceramidase/GBA

Glucosylceramidase is a lysosomal enzyme that cleaves the beta-glucosidic linkage of glucosylceramide (1, 2), an intermediate in glycolipid metabolism. The mature enzyme has 497 amino acids with a molecular weight of 62 kDa (3). Glycosylation occurs at four of five N-glycosylation sites and is essential for the trafficking and activity of the enzyme (4). The enzyme is activated in lysosomes by saposin C, although the mechanism of activation is not well understood (5). Defects in Glucosylceramidase are the cause of Gaucher disease, also known as glucocerebrosidase deficiency (6). Gaucher disease is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Symptoms of Gaucher disease may include enlarged spleen and liver, liver malfunction, skeletal disorders and bone lesions, severe neurologic complications, swelling of lymph nodes, anemia, low blood platelets and yellow fatty deposits on the white of the eye (7). Currently, enzyme replacement therapy is used to treat patients with the disease (8, 9).

References
  1. Sorge, J. et al. (1985) Proc. Natl. Acad. Sci. USA 82:7289.
  2. Ginns, E. I. et al. (1984) Biochem. Biophyl. Res. Commun. 123:574.
  3. Horowitz, M. et al. (1989) Genomics 4:87.
  4. Grace, M.E. et al. (1994) J. Biol. Chem. 269:2283.
  5. Bruhn, h. (2005) Biochem. J. 389:249.
  6. Liou, B. et al. (2006) J. Biol. Chem. 281:4242.
  7. Grabowski, G.A. (2008). Lancet 372:1263.
  8. Zheng, W. et al. (2007) Proc. Natl. Acad. Sci. USA 104:13192.
  9. Beutler, E. and Gelbart, T. (1996) Hum. Mutat. 8:207.
Entrez Gene IDs
2629 (Human); 14466 (Mouse)
Alternate Names
Acid beta-glucosidase; Alglucerase; beta-glucocerebrosidase; D-glucosyl-N-acylsphingosine glucohydrolase; EC 3.2.1.45; GBA; GBA1; GC; GCB; GLUC; glucosidase, beta, acid; glucosidase, beta; acid (includes glucosylceramidase); Glucosylceramidase; Imiglucerase; lysosomal glucocerebrosidase

Citations for Recombinant Human Glucosylceramidase/GBA Protein, CF

R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions.

11 Citations: Showing 1 - 10
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  1. Reduced progranulin increases tau and ?-synuclein inclusions and alters mouse tauopathy phenotypes via glucocerebrosidase
    Authors: Takahashi, H;Bhagwagar, S;Nies, SH;Ye, H;Han, X;Chiasseu, MT;Wang, G;Mackenzie, IR;Strittmatter, SM;
    Nature communications
    Species: Mouse
    Sample Types: Whole Cells
    Applications: Bioassay
  2. The SATB1-MIR22-GBA axis mediates glucocerebroside accumulation inducing a cellular senescence-like phenotype in dopaminergic neurons
    Authors: Russo, T;Kolisnyk, B;Bs, A;Kim, TW;Martin, J;Plessis-Belair, J;Ni, J;Pearson, JA;Park, EJ;Sher, RB;Studer, L;Riessland, M;
    bioRxiv : the preprint server for biology
    Species: Human
    Sample Types: Transfected Whole Cells, Whole Cells
    Applications: Bioassay
  3. Recapture Lysosomal Enzyme Deficiency via Targeted Gene Disruption in the Human Near-Haploid Cell Line HAP1
    Authors: A Brown, J Zhang, B Lawler, B Lu
    Genes, 2021-07-15;12(7):.
    Species: Human
    Sample Types:
    Applications: Cell Culture
  4. Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor
    Authors: MC Babcock, CR Mikulka, B Wang, S Chandriani, S Chandra, Y Xu, K Webster, Y Feng, HR Nelvagal, A Giaramita, BK Yip, M Lo, X Jiang, Q Chao, JC Woloszynek, Y Shen, S Bhagwat, MS Sands, BE Crawford
    Scientific Reports, 2021-07-14;11(1):14486.
    Species: Human
    Sample Types: Recombinant Protein
    Applications: Bioassay
  5. Validation and assessment of preanalytical factors of a fluorometric in vitro assay for glucocerebrosidase activity in human cerebrospinal fluid
    Authors: L Oftedal, J Maple-Grød, MGG Førland, G Alves, J Lange
    Scientific Reports, 2020-12-16;10(1):22098.
    Species: Human
    Sample Types: CSF
    Applications: Cell Culture
  6. Targeted delivery of lysosomal enzymes to the endocytic compartment in human cells using engineered extracellular vesicles
    Authors: MA Do, D Levy, A Brown, G Marriott, B Lu
    Sci Rep, 2019-11-21;9(1):17274.
    Species: N/A
    Sample Types: Protein
    Applications: Enzyme Assay
  7. Mitochondrial dysfunction and mitophagy defect triggered by heterozygous GBA mutations
    Authors: H Li, A Ham, TC Ma, SH Kuo, E Kanter, D Kim, HS Ko, Y Quan, SP Sardi, A Li, O Arancio, UJ Kang, D Sulzer, G Tang
    Autophagy, 2018-10-12;15(1):113-130.
    Species: Mouse
    Sample Types: Tissue Homogenates
  8. ?-Synuclein accumulation and GBA deficiency due to L444P GBA mutation contributes to MPTP-induced parkinsonism
    Authors: SP Yun, D Kim, S Kim, S Kim, SS Karuppagou, SH Kwon, S Lee, TI Kam, S Lee, S Ham, JH Park, VL Dawson, TM Dawson, Y Lee, HS Ko
    Mol Neurodegener, 2018-01-08;13(1):1.
    Species: Mouse
    Sample Types: Whole Cells
    Applications: Bioassay
  9. Fluorescence-Quenched Substrates for Quantitative Live Cell Imaging of Glucocerebrosidase Activity
    Authors: RA Ashmus, DL Shen, DJ Vocadlo
    Meth. Enzymol., 2017-10-31;598(0):199-215.
    Species: Human
    Sample Types:
    Applications: Bioassay
  10. Lysosomal Enzyme Glucocerebrosidase Protects against Abeta1-42 Oligomer-Induced Neurotoxicity.
    Authors: Choi S, Kim D, Kam T, Yun S, Kim S, Park H, Hwang H, Pletnikova O, Troncoso J, Dawson V, Dawson T, Ko H
    PLoS ONE, 2015-12-02;10(12):e0143854.
    Species: Human
    Sample Types: Tissue Homogenates
    Applications: Bioassay
  11. Tool compounds robustly increase turnover of an artificial substrate by glucocerebrosidase in human brain lysates.
    Authors: Berger, Zdenek, Perkins, Sarah, Ambroise, Claude, Oborski, Christin, Calabrese, Matthew, Noell, Stephen, Riddell, David, Hirst, Warren D
    PLoS ONE, 2015-03-12;10(3):e0119141.
    Species: Human, Mouse
    Sample Types: Tissue Homogenates
    Applications: Enzyme Assay

FAQs

  1. Why is DTT (dithiothreitol) added to the assay buffer in the assay described for Recombinant Human Glucosylceramidase/GBA Protein, CF, Catalog # 7410-GHB?

    • The 5 mM DTT is added to the assay buffer in the assay protocol for catalog #  7410-GHB to prevent interchain disulfide bond formation that can cause aggregation of the protein and loss of enzyme activity over time. 

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