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Key Documents

WH0002629M1

Sigma-Aldrich

Monoclonal Anti-GBA antibody produced in mouse

clone 2e2, purified immunoglobulin, buffered aqueous solution

Sinonimo/i:

Anti-GBA1, Anti-GCB, Anti-GLUC, Anti-glucosidase, beta; acid (includes glucosylceramidase)

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About This Item

Codice UNSPSC:
12352203
NACRES:
NA.41

Origine biologica

mouse

Coniugato

unconjugated

Forma dell’anticorpo

purified immunoglobulin

Tipo di anticorpo

primary antibodies

Clone

2e2, monoclonal

Forma fisica

buffered aqueous solution

Reattività contro le specie

human

tecniche

immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
indirect ELISA: suitable
indirect immunofluorescence: suitable
western blot: 1-5 μg/mL

Isotipo

IgG2aκ

N° accesso Genebanck

N° accesso UniProt

Condizioni di spedizione

dry ice

Temperatura di conservazione

−20°C

modifica post-traduzionali bersaglio

unmodified

Informazioni sul gene

human ... GBA(2629)

Categorie correlate

Descrizione generale

GBA (β-glucocerebrosidase) gene is mapped to human chromosome 1q21. It is a lysosomal enzyme and is widely expressed. The protein has domain I with three‐stranded anti‐parallel β‐sheets, domain II with two β‐sheets making an immunoglobulin‐like domain and domain III with eight‐stranded β/αtriosephosphate isomerase (TIM) barrel.
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)

Immunogeno

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

Applicazioni

Monoclonal Anti-GBA antibody has been used in immunofluorescence staining.

Azioni biochim/fisiol

GBA (β-glucocerebrosidase) is responsible for the conversion of the glycolipid glucocerebroside to ceramide and glucose. Mutations in this gene are associated with Gaucher disease and Parkinson disease.

Stato fisico

Solution in phosphate buffered saline, pH 7.4

Note legali

GenBank is a registered trademark of United States Department of Health and Human Services

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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Raccomandato

Codice della classe di stoccaggio

10 - Combustible liquids

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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Stefania Zampieri et al.
International journal of molecular sciences, 22(11) (2021-06-03)
Gaucher disease (GD) is an autosomal recessive lysosomal disorder due to beta-glucosidase gene (GBA) mutations. The molecular diagnosis of GD is complicated by the presence of recombinant alleles originating from a highly homologous pseudogene. Clinical exome sequencing (CES) is a
The relationship between glucocerebrosidase mutations and Parkinson disease
Migdalska-Richards A and Schapira AH
Journal of Neurochemistry (2016)
Altered Differentiation Potential of Gaucher?s Disease iPSC Neuronal Progenitors due to Wnt/b-Catenin Downregulation
Awad O, et al.
Stem Cell Reports, 9 (2017)
Rodolfo Tonin et al.
Journal of neurology, 266(1), 92-101 (2018-11-02)
Patients with Gaucher Disease (GD) exhibit three phenotypes, including type 1 (non-neuronopathic), type 2 (acute neuronopathic), and type 3 (subacute neuronopathic). Identifying which GBA changes represent benign polymorphisms and which may result in disease-causing mutations is essential for diagnosis and
Cathrine K Fog et al.
EBioMedicine, 38, 142-153 (2018-12-01)
Gaucher Disease is caused by mutations of the GBA gene which encodes the lysosomal enzyme acid beta-glucosidase (GCase). GBA mutations commonly affect GCase function by perturbing its protein homeostasis rather than its catalytic activity. Heat shock proteins are well known

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