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- 详细信息
- 技术资料
- 免疫原:
Peptide derived from Hu GLCNE.
- 亚型:
Rabbit Polyclonal IgG
- 形态:
液态
- 保存条件:
保存于-20℃
- 克隆性:
多克隆
- 标记物:
无标记(PLlabs可供应生物素、酶、荧光素标记的抗体)
- 适应物种:
Hu, Mo, Rat, Pig, Cow, Hor, Sheep,
- 保质期:
按说明书条件可保存1-2年
- 抗原来源:
见详细说明资料
- 目录编号:
PL0300020
- 级别:
超纯
- 库存:
大量
- 供应商:
华拓生物
- 宿主:
Rabbit
- 应用范围:
WB: 1:100-500 E: 1:500-1000 IHC: 1:100-500 IF: 1:50-200
- 浓度:
1mg/ml
- 靶点:
GLCNE
- 抗体英文名:
GLCNE Antibody
- 抗体名:
GLCNE蛋白抗体
- 规格:
0.1ml
GLCNE Antibody
Cat#:PL0300020
Isotype:Rabbit Polyclonal IgG
Immunogen:Peptide derived from Hu GLCNE.
Purification: Immunogen affinity purified
Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)
Background:
Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)
Background:
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.
Alternate Names:
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.
Specificity Reaction:Hu, Mo, Rat, Pig, Cow, Hor, Sheep,
Application Notes:WB: 1:100-500 E: 1:500-1000 IHC: 1:100-500 IF: 1:50-200
Storage :
Store at –20ºC for one year. Avoid repeated freeze-thaw cycles. At 4ºC for 2-3 weeks after reconstituted.
This product for research use only, not for use in humans, therapeutic or diagnostic procedures.
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GLCNE Antibody
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