G4171

Anti-Glucocerebrosidase (C-terminal) antibody produced in rabbit

~1 mg/mL, affinity isolated antibody, buffered aqueous solution

• Synonim(y): Anti-D-glucosyl-N-acylsphingosine glucohydrolase, Anti-GBA, Anti-GBA1, Anti-Glucosidase, beta (Gluc), Anti-Glucosylceramidase (GlcCerase), Anti-Lysosomal glucocerebrosidase
• NACRES: NA.41
• UNSPSC Code: 12352203
• Conjugate: unconjugated
• Clone: polyclonal
• Application: WB
• Citations: 62

Właściwości

• biological source: rabbit
• Quality Segment: 200
• 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.: P04062
• shipped in: dry ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... GBA(2629) ; mouse ... Gba(14466)

Opis

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.

Other Notes

2024 CiteAb Award Winner for Supplier Succeeding in Parkinson′s Research

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.

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