G4171
Anti-Glucocerebrosidase (C-terminal) antibody produced in rabbit
~1 mg/mL, affinity isolated antibody, buffered aqueous solution
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Synonym(s):
Anti-D-glucosyl-N-acylsphingosine glucohydrolase, Anti-GBA, Anti-GBA1, Anti-Glucosidase, beta (Gluc), Anti-Glucosylceramidase (GlcCerase), Anti-Lysosomal glucocerebrosidase
UNSPSC Code:
12352203
NACRES:
NA.41
Recommended Products
biological source
rabbit
Quality Level
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
antigen ~60 kDa
species reactivity
human, mouse, rat
packaging
antibody small pack of 25 μL
enhanced validation
recombinant expression
Learn more about Antibody Enhanced Validation
concentration
~1 mg/mL
technique(s)
western blot: 1-2 μg/mL using HEK293-T cells lysate expressing human glucocerebrosidase (GBA)
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... GBA(2629)
mouse ... Gba(14466)
Related Categories
General description
Glucocerebrosidase is an enzyme having glucosylceramidase activity. Defect in lysosomal hydrolase glucocerebrosidase results in Gaucher disease. Mutation in GBA will retard or block the transport of GBA to endoplasmic reticulum. Anti-glucocerebrosidase (C-terminal) antibody can be used to incubate the SDS-PAGE gel. Anti-Glucocerebrosidase antibody reacts specifically with human GBA.
Immunogen
synthetic peptide corresponding to amino acids 517-536 of human glucocerebrosidase (GBA), conjugated to KLH. This sequence is identical in rat GBA and highly conserved in mouse GBA (single amino acid substitution).
Application
Anti-Glucocerebrosidase (C-terminal) antibody produced in rabbit has been used in
- immunohistochemistry
- immunoblot analysis
Anti-glucocerebrosidase (C-terminal) antibody can be used in western blotting and immunoblotting.
Biochem/physiol Actions
Glucocerebrosidase (GBA) activity is reduced in human with mutations in GBA gene and causes accumulation of glucosylceramide (GlcCer). Fibroblasts from patients with defined GBA mutations show either retarded or blocked transport of GBA in the endoplasmic reticulum. Mutations in the human GBA gene may contribute to the development of common age-related dementia known as dementia with Lewy bodies. Several studies indicate that mutations in the human GBA gene are associated with early-onset Parkinson disease.
Glucocerebrosidase is an enzyme having glucosylceramidase activity. Defect in lysosomal hydrolase glucocerebrosidase results in Gaucher disease. Mutation in GBA will retard or block the transport of GBA to endoplasmic reticulum.
Physical form
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
recommended
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WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Personal Protective Equipment
dust mask type N95 (US), Eyeshields, Gloves
Regulatory Information
常规特殊物品
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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Find documentation for the products that you have recently purchased in the Document Library.
Gaucher disease paradigm: From ERAD to comorbidity.
Inna Bendikov-Bar, Mia Horowitz
Human Mutation, 33(10), doi: 10-doi: 10 (2012)
Reducing GBA2 activity ameliorates neuropathology in Niemann-Pick type C mice
Marques ARA, et al.
Testing, 10(8), e0135889-e0135889 (2015)
Inna Bendikov-Bar et al.
Blood cells, molecules & diseases, 50(2), 141-145 (2012-11-20)
Gaucher disease (GD) is characterized by accumulation of glucosylceramide in lysosomes due to mutations in the GBA1 gene encoding the lysosomal hydrolase β-glucocerebrosidase (GCase). The disease has a broad spectrum of phenotypes, which were divided into three different Types; Type
N370S-GBA1 mutation causes lysosomal cholesterol accumulation in Parkinson's disease
Garcia-Sanz P, et al.
Movement Disorders, 32(10), 1409-1422 (2017)
Eun-Jin Bae et al.
Nature communications, 5, 4755-4755 (2014-08-27)
Deposition of α-synuclein aggregates occurs widely in the central and peripheral nervous systems in Parkinson's disease (PD). Although recent evidence has suggested that cell-to-cell transmission of α-synuclein aggregates is associated with the progression of PD, the mechanism by which α-synuclein
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