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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
CSB-EP017315HUb3 IHC-1

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
CSB-EP017315HUb3 IHC-2

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)

GAA Proteins

GAA Proteins for Homo sapiens (Human)

GAA ELISA Kit

GAA ELISA Kit for Homo sapiens (Human)