BD-PB4329-GAA Rabbit Polyclonal Antibody (N-term)-现货抗体产品库武汉枢密脑科学技术有限公司

GAA Rabbit Polyclonal Antibody (N-term)

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产品基本信息

产品货号

BD-PB4329

产品名称

GAA Rabbit Polyclonal Antibody (N-term)

别名

Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA

类别

常规抗体

推荐应用

反应种属

Human, Rat, Mouse

存储缓冲液

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) New type preservative N. This antibody is purified through a protein A column, followed by peptide affinity purification.

Human Gene ID

NP_000143.2;NP_001073271.1;NP_001073272.1

Human Swissprot No.

P10253

特异性

This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.

稀释度

IHC-P-Leica~~1:500;WB~~1:1000

预测分子量

105kDa

运输及保存条件

Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

宿主

Rabbit

同种型

Rabbit IgG

背景介绍

This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.

细胞定位

Lysosome. Lysosome membrane

功能

Essential for the degradation of glycogen in lysosomes (PubMed:1856189, PubMed:7717400, PubMed:14695532, PubMed:18429042). Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans (PubMed:29061980).

期货

现货

Immunohistochemical analysis of paraffin-embedded Human placenta tissue using BD-PB4329 performed on the Leica® BOND RXm. Tissue was fixed with formaldehyde at room temperature, antigen retrieval was by heat mediation with a EDTA buffer (pH9. 0). Samples were incubated with primary antibody(1:500) for 1 hours at room temperature. A undiluted biotinylated CRF Anti-Polyvalent HRP Polymer antibody was used as the secondary antibody.

Immunohistochemical analysis of paraffin-embedded Human liver tissue using BD-PB4329 performed on the Leica® BOND RXm. Tissue was fixed with formaldehyde at room temperature, antigen retrieval was by heat mediation with a EDTA buffer (pH9. 0). Samples were incubated with primary antibody(1:500) for 1 hours at room temperature. A undiluted biotinylated CRF Anti-Polyvalent HRP Polymer antibody was used as the secondary antibody.

All lanes : Anti-GAA Antibody (N-term) at 1:1000 dilution Lane 1: Hela whole cell lysate Lane 2: MCF-7 whole cell lysate Lane 3: SW620 whole cell lysate Lane 4: A549 whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 105 kDa Blocking/Dilution buffer: 5% NFDM/TBST.

Anti-GAA Antibody (N-term) at 1:1000 dilution + Human placenta tissue lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 105 kDa Blocking/Dilution buffer: 5% NFDM/TBST.