AGA Antibody
-
中文名稱：AGA兔多克隆抗體
-
貨號：CSB-PA213113
-
規格：￥1100
-
圖片：
-
Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane: Raji cells, Primary antibody: CSB-PA213113(AGA Antibody) at dilution 1/125, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 30 seconds
-
-
其他：
產品詳情
-
Uniprot No.：
-
基因名：AGA
-
別名：Aga antibody; AGU antibody; Aspartylglucosaminidase antibody; Aspartylglucosylamine deaspartylase antibody; Aspartylglycosaminuria antibody; ASPG_HUMAN antibody; ASRG antibody; GA antibody; Glycosylasparaginase antibody; Glycosylasparaginase beta chain antibody; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase antibody; N4 (N acetyl beta glucosaminyl) L asparagine amidase antibody; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase antibody
-
宿主：Rabbit
-
反應種屬：Human,Mouse,Rat
-
免疫原：Fusion protein of Human AGA
-
免疫原種屬：Homo sapiens (Human)
-
標記方式：Non-conjugated
-
抗體亞型：IgG
-
純化方式：Antigen affinity purification
-
濃度：It differs from different batches. Please contact us to confirm it.
-
保存緩沖液：-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
-
產品提供形式：Liquid
-
應用范圍：ELISA,WB
-
推薦稀釋比：
Application Recommended Dilution ELISA 1:1000-1:2000 WB 1:200-1:1000 -
Protocols：
-
儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
-
貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
-
用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
-
功能：Cleaves the GlcNAc-Asn bond which joins oligosaccharides to the peptide of asparagine-linked glycoproteins.
-
基因功能參考文獻：
- 1.8A resolution crystal structure of mature G172D mutant of a model missense GA corresponding to a Canadian aspartylglucosaminuria allele; studied the effect of its single amino acid change on substrate processing PMID: 28457719
- We show that gene-silenced cells show specifically reduced AGA activity and store globotriaosylceramide. In gene-silenced cells, release of the neurotransmitter acetylcholine is significantly reduced, demonstrating that this model may be used to study specific neuronal functions such as neurotransmitter release in Fabry disease PMID: 27471012
- study reports 2 novel aspartylglucosaminidase gene mutations, one in Qatari twins with an early, perinatal presentation not previously described for aspartylglucosaminuria and the other in 3 Turkish children with newly diagnosed aspartylglucosaminuria and a more classical disease course PMID: 23271757
- [review] Natural killer (NK) cell tumors, subtypes of myeloid leukemias and T-cell lymphomas respond to ASNase; ovarian carcinomas and other solid tumors have been proposed as additional targets for ASNase, with a potential role for glutaminase. activity. PMID: 21854356
- Molecular mechanism for the autoproteolytic activation of aspartylglucosaminidase. PMID: 14616088
- A new point mutation, c.44T>G, found in a Finnish compound heterozygote causes a L15R AA substitution in the signal sequence of the AGA enzyme, affecting AGA translocation by altering a critical hydrophobic core structure in the signal sequence. PMID: 15365992
- aspartylglucosaminidase may have a role in development of congenital disorders of glycosylation type I PMID: 16435229
- The amino acid substitutions in aspartylglucosaminidase responsible for aspartylglucosaminuria were classified and divided in three groups. PMID: 18992224
- Increased AGA plasma activity, although a consistent finding in congenital disorders of glycosylation patients, is not specific to this group of disorders. PMID: 19100247
顯示更多
收起更多
-
相關疾?。?/div>Aspartylglucosaminuria (AGU)亞細胞定位：Lysosome.蛋白家族：Ntn-hydrolase family數據庫鏈接：
Most popular with customers
-
-
YWHAB Recombinant Monoclonal Antibody
Applications: ELISA, WB, IHC, IF, FC
Species Reactivity: Human, Mouse, Rat
-
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody
Applications: ELISA, WB, IHC
Species Reactivity: Human
-
-
-
-
-
