| 克隆类型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from Hu GLCNE. |
|
纯化方式 ： |
Immunogen affinity purified |
|
缓 冲 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
产品描述 ： |
The bifunctional enzyme UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE/Mnk), or GLCNE, regulates and initiates biosynthesis of N-acetylneuraminic acid (NeuAc), a precursor of sialic acids. GLCNE is required for normal sialylation in hematopoietic cells. Sialylation is implicated in cell adhesion, signal transduction, tumorigenicity and metastatic behavior of malignant cells. It is upregulated after PKC-dependent phosphorylation and is most abundantly expressed in liver and placenta. It is also expressed, to a lesser extent, in heart, brain, lung, kidney, skeletal muscle and pancreas. Defects in GLCNE are the cause of sialuria, inclusion body myopathy 2 (IBM2) and Nonaka myopathy (NM) or distal myopathy with rimmed vacuoles (DMRV). Sialuria is an autosomal dominant disorder caused by a lack of feedback inhibition of GLCNE by CMP-NeuAc, resulting in overproduction of NeuAc. It is characterized by an accumulation of free sialic acid in the cytoplasm and large quantities of neuraminic acid in the urine. |
| 适用物种 ： |
Hu, Mo, Rat, Pig, Cow, Hor, Sheep, |
| 应用说明 ： |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:50-1:200 |
| 别 名 ： |
IBM2; Uae1; Bifunctional UDP N acetylglucosamine 2 epimerase/N acetylmannosamine kinase; DMRV; ManAc kinase; N acylmannosamine kinase; NM; RP23-209M8.6; UDP GlcNAc 2 epimerase; UDP GlcNAc 2 epimerase/ManAc kinase; Uridine diphosphate N acetylglucosamine 2 epimerase; GLCNE_Hu. |
| 保存条件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 标 记 物 ： |
PLLABS公司可供应生物素、辣根过氧化物酶、荧光素标记抗体 |
|
产品说明 ： |
|
|
