GBA monoclonal antibody (M01), clone 2E2
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More Files
- More Functions
Western Blot (Cell lysate)
GBA monoclonal antibody (M01), clone 2E2 Western Blot analysis of GBA expression in MCF-7 ( Cat # L046V1 ).
Western Blot (Transfected lysate)
Western Blot analysis of GBA expression in transfected 293T cell line by GBA monoclonal antibody (M01), clone 2E2.
Lane 1: GBA transfected lysate(60 KDa).
Lane 2: Non-transfected lysate.
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunoperoxidase of monoclonal antibody to GBA on formalin-fixed paraffin-embedded human breast cancer. [antibody concentration 3 ug/ml]
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged GBA is approximately 1ng/ml as a capture antibody.
Immunofluorescence
Immunofluorescence of monoclonal antibody to GBA on HeLa cell. [antibody concentration 10 ug/ml]
Western Blot detection against Immunogen (35.64 KDa) .
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Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant GBA.
Immunogen
GBA (NP_000148, 146 a.a. ~ 235 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
SYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGS
Host
Mouse
Reactivity
Human
Interspecies Antigen Sequence
Mouse (84); Rat (88)
Isotype
IgG2a Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (35.64 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Cell lysate)
GBA monoclonal antibody (M01), clone 2E2 Western Blot analysis of GBA expression in MCF-7 ( Cat # L046V1 ).Western Blot (Transfected lysate)
Western Blot analysis of GBA expression in transfected 293T cell line by GBA monoclonal antibody (M01), clone 2E2.
Lane 1: GBA transfected lysate(60 KDa).
Lane 2: Non-transfected lysate.
Western Blot (Recombinant protein)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunoperoxidase of monoclonal antibody to GBA on formalin-fixed paraffin-embedded human breast cancer. [antibody concentration 3 ug/ml]Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged GBA is approximately 1ng/ml as a capture antibody.ELISA
Immunofluorescence
Immunofluorescence of monoclonal antibody to GBA on HeLa cell. [antibody concentration 10 ug/ml] -
Gene Info — GBA
Entrez GeneID
2629GeneBank Accession#
NM_000157Protein Accession#
NP_000148Gene Name
GBA
Gene Alias
GBA1, GCB, GLUC
Gene Description
glucosidase, beta; acid (includes glucosylceramidase)
Gene Ontology
HyperlinkGene Summary
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 encoding the same protein. [provided by RefSeq
Other Designations
D-glucosyl-N-acylsphingosine glucohydrolase|OTTHUMP00000033992|OTTHUMP00000033993|glucocerebrosidase|glucocerebrosidase (alt.)|lysosomal glucocerebrosidase
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Interactome
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Pathway
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Disease
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Publication Reference
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Inhibition of cysteine protease cathepsin Lincreases the level and activity of lysosomal glucocerebrosidase.
Myung Jong Kim, Soojin Kim, Thomas Reinheckel, Dimitri Krainc.
JCI Insight 2024 Feb; 9(3):e169594.
Application：IF, Human, Fibroblast, HEK293-FT cells.
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Production of recombinant human acid β-glucosidase with high mannose-type N-glycans in rice gnt1 mutant for potential treatment of Gaucher disease.
Jung JW, Choi HY, Huy NX, Park H, Kim HH, Yang MS, Kang SH, Kim DI, Kim NS.
Protein Expression and Purification 2019 Feb; 158:81.
Application：WB-Re, Mouse, Recombinant proteins from transgenic rice suspension cell culture medium.
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Reduced glucocerebrosidase activity in monocytes from patients with Parkinson's disease.
Atashrazm F, Hammond D, Perera G, Dobson-Stone C, Mueller N, Pickford R, Kim WS, Kwok JB, Lewis SJG, Halliday GM, Dzamko N.
Scientific Reports 2018 Oct; 8(1):15446.
Application：WB-Tr, Human, HEK 293 cells, Lymphocytes, Monocytes.
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Parkin-mediated ubiquitination of mutant glucocerebrosidase leads to competition with its substrates PARIS and ARTS.
Bendikov-Bar I, Rapaport D, Larisch S, Horowitz M.
Orphanet Journal of Rare Diseases 2014 Jun; 9:86.
Application：IP, WB-Ce, Human, Skin fibroblasts.
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Reduced glucocerebrosidase is associated with increased α-synuclein in sporadic Parkinson's disease.
Murphy KE, Gysbers AM, Abbott SK, Tayebi N, Kim WS, Sidransky E, Cooper A, Garner B, Halliday GM.
Brain : A Journal of Neurology. 2014 Mar; 137(Pt 3):834.
Application：WB-Ti, Human, Brain.
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Gaucher disease paradigm: from ERAD to comorbidity.
Bendikov-Bar I, Horowitz M.
Human Mutation 2012 Oct; 33(10):1398.
Application：WB-Ce, Human, Human skin fibroblasts.
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Characterization of the ERAD process of the L444P mutant glucocerebrosidase variant.
Bendikov-Bar I, Ron I, Filocamo M, Horowitz M.
Blood Cells, Molecules & Diseases 2011 Jan; 46(1):4.
Application：WB-Ce, Human, Fibroblasts.
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Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome.
Campeau PM, Rafei M, Boivin MN, Sun Y, Grabowski GA, Galipeau J.
Blood 2009 Oct; 114(15):3181.
Application：WB, Human, Mesenchymal stromal cells.
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Inhibition of cysteine protease cathepsin Lincreases the level and activity of lysosomal glucocerebrosidase.
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