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Key Documents

G4171

Sigma-Aldrich

Anti-Glucocerebrosidase (C-terminal)

enhanced validation

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

Sinonimo/i:

Anti-D-glucosyl-N-acylsphingosine glucohydrolase, Anti-GBA, Anti-GBA1, Anti-Glucosidase, beta (Gluc), Anti-Glucosylceramidase (GlcCerase), Anti-Lysosomal glucocerebrosidase

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About This Item

Codice UNSPSC:
12352203
NACRES:
NA.41

Origine biologica

rabbit

Livello qualitativo

Coniugato

unconjugated

Forma dell’anticorpo

affinity isolated antibody

Tipo di anticorpo

primary antibodies

Clone

polyclonal

Forma fisica

buffered aqueous solution

PM

antigen ~60 kDa

Reattività contro le specie

human, mouse, rat

Confezionamento

antibody small pack of 25 μL

Convalida avanzata

recombinant expression
Learn more about Antibody Enhanced Validation

Concentrazione

~1 mg/mL

tecniche

western blot: 1-2 μg/mL using HEK293-T cells lysate expressing human glucocerebrosidase (GBA)

N° accesso UniProt

Condizioni di spedizione

dry ice

Temperatura di conservazione

−20°C

modifica post-traduzionali bersaglio

unmodified

Informazioni sul gene

human ... GBA(2629)
mouse ... Gba(14466)

Descrizione generale

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.

Immunogeno

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).

Applicazioni

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.

Azioni biochim/fisiol

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.

Stato fisico

Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

Esclusione di responsabilità

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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Codice della classe di stoccaggio

12 - Non Combustible Liquids

Classe di pericolosità dell'acqua (WGK)

WGK 1

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable

Dispositivi di protezione individuale

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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Reducing GBA2 activity ameliorates neuropathology in Niemann-Pick type C mice
Marques ARA, et al.
Testing, 10(8), e0135889-e0135889 (2015)
Gaucher disease paradigm: From ERAD to comorbidity.
Inna Bendikov-Bar, Mia Horowitz
Human Mutation, 33(10), doi: 10-doi: 10 (2012)
Joseph R Mazzulli et al.
The Journal of neuroscience : the official journal of the Society for Neuroscience, 36(29), 7693-7706 (2016-07-23)
Parkinson's disease (PD) is characterized by the accumulation of α-synuclein (α-syn) within Lewy body inclusions in the nervous system. There are currently no disease-modifying therapies capable of reducing α-syn inclusions in PD. Recent data has indicated that loss-of-function mutations in
Nahid Tayebi et al.
Molecular genetics and metabolism, 122(4), 198-208 (2017-11-28)
Mutations in GBA1 encountered in Gaucher disease are a leading risk factor for Parkinson disease and associated Lewy body disorders. Many GBA1 mutation carriers, especially those with severe or null GBA1 alleles, have earlier and more progressive parkinsonism. To model
Inna Bendikov-Bar et al.
Orphanet journal of rare diseases, 9, 86-86 (2014-06-18)
Parkinson's disease (PD) is a movement neurodegenerative disorder characterized by death of dopaminergic neurons in the substantia nigra pars compacta of the brain that leads to movement impairments including bradykinesia, resting tremor, postural instability and rigidity. Mutations in several genes

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