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Merck
CN

WH0002629M1

Monoclonal Anti-GBA antibody produced in mouse

clone 2e2, purified immunoglobulin, buffered aqueous solution

别名:

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

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关于此项目

NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
2e2, monoclonal
Application:
ELISA (i), IF, IHC (p), WB
Citations:
4
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biological source

mouse

Quality Level

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

2e2, monoclonal

form

buffered aqueous solution

species reactivity

human

technique(s)

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

isotype

IgG2aκ

GenBank accession no.

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... GBA(2629)

General description

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)

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

Application

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

Biochem/physiol Actions

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.

Physical form

Solution in phosphate buffered saline, pH 7.4

Legal Information

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

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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存储类别

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

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

法规信息

低风险生物材料
常规特殊物品

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历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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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)
The relationship between glucocerebrosidase mutations and Parkinson disease
Migdalska-Richards A and Schapira AH
Journal of Neurochemistry (2016)
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



全球贸易项目编号

货号GTIN
WH0002629M1-100UG04061832742489