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 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)
產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 種屬反應(yīng)性 | 應(yīng)用類型 |
---|---|---|---|
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)
產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 來(lái)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)
產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 樣本類型 | 靈敏度 |
---|---|---|---|
CSB-EL009125HU | Human Lysosomal alpha-glucosidase(GAA) ELISA kit | serum, plasma, tissue homogenates, cell lysates | 6.25 pg/mL |