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GAA

GAA,又被稱為LYAG(Lysosomal alpha-glucosidase),溶酶體α-葡糖苷酶是一種關鍵的酶,它在溶酶體中發揮重要作用,參與糖原的分解過程,確保糖原被正確降解為葡萄糖分子以維持細胞能量代謝和其他生物過程的正常進行。然而,當溶酶體α-葡糖苷酶出現缺陷或功能失調時,會引發一系列遺傳性疾病,其中最具代表性的是龐貝病。龐貝病是一種由該酶缺乏或功能不足導致的常染色體隱性遺傳病,表現為糖原在溶酶體內積累,引發肌無力、心臟肥大等臨床癥狀。

熱銷產品

GAA Recombinant Monoclonal Antibody

驗證數據

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)



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