Your Good Partner in Biology Research

GAA

GAA,又被稱為L(zhǎng)YAG(Lysosomal alpha-glucosidase),溶酶體α-葡糖苷酶是一種關(guān)鍵的酶,它在溶酶體中發(fā)揮重要作用,參與糖原的分解過(guò)程,確保糖原被正確降解為葡萄糖分子以維持細(xì)胞能量代謝和其他生物過(guò)程的正常進(jìn)行。然而,當(dāng)溶酶體α-葡糖苷酶出現(xiàn)缺陷或功能失調(diào)時(shí),會(huì)引發(fā)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(dǎo)致的常染色體隱性遺傳病,表現(xiàn)為糖原在溶酶體內(nèi)積累,引發(fā)肌無(wú)力、心臟肥大等臨床癥狀。

熱銷產(chǎn)品

GAA Recombinant Monoclonal Antibody

驗(yàn)證數(shù)據(jù)

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)