GAA
GAA,又被称为LYAG(Lysosomal alpha-glucosidase),溶酶体α-葡糖苷酶是一种关键的酶,它在溶酶体中发挥重要作用,参与糖原的分解过程,确保糖原被正确降解为葡萄糖分子以维持细胞能量代谢和其他生物过程的正常进行。然而,当溶酶体α-葡糖苷酶出现缺陷或功能失调时,会引发一系列遗传性疾病,其中最具代表性的是庞贝病。庞贝病是一种由该酶缺乏或功能不足导致的常染色体隐性遗传病,表现为糖原在溶酶体内积累,引发肌无力、心脏肥大等临床症状。
热销产品
GAA Recombinant Monoclonal Antibody
验证数据
IHC image of CSB-RA566370A0HU diluted at 1:50 and staining in paraffin-embedded human breast cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.15% DAB.
IHC image of CSB-RA566370A0HU diluted at 1:50 and staining in paraffin-embedded human placenta tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.15% DAB.
GAA Antibodies
GAA for Homo sapiens (Human)
产品货号 | 产品名称 | 种属反应性 | 应用类型 |
---|---|---|---|
CSB-PA009125GA01HU | GAA Antibody | Human,Mouse,Rat | ELISA,WB,IHC |
CSB-PA009125LA01HU | GAA Antibody | Human, Mouse | ELISA, WB, IHC, IF |
CSB-PA009125LB01HU | GAA Antibody, HRP conjugated | Human | ELISA |
CSB-PA009125LC01HU | GAA Antibody, FITC conjugated | Human | |
CSB-PA009125LD01HU | GAA Antibody, Biotin conjugated | Human | ELISA |
CSB-RA566370A0HU | GAA Recombinant Monoclonal Antibody | Human | ELISA, IHC |
GAA Proteins
GAA Proteins for Homo sapiens (Human)
产品货号 | 产品名称 | 来源 |
---|---|---|
CSB-YP009125HU CSB-EP009125HU CSB-BP009125HU CSB-MP009125HU CSB-EP009125HU-B |
Recombinant Human Lysosomal alpha-glucosidase (GAA), partial | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
GAA ELISA Kit
GAA ELISA Kit for Homo sapiens (Human)
产品货号 | 产品名称 | 样本类型 | 灵敏度 |
---|---|---|---|
CSB-EL009125HU | Human Lysosomal alpha-glucosidase(GAA) ELISA kit | serum, plasma, tissue homogenates, cell lysates | 6.25 pg/mL |