GNE 抗体 (AbBy Fluor® 647)
Quick Overview for GNE 抗体 (AbBy Fluor® 647) (ABIN1405218)
抗原
See all GNE 抗体适用
宿主
克隆类型
标记
应用范围
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交叉反应
- 人, 小鼠, 大鼠
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human GLCNE
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亚型
- IgG
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anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) (AA 1-150) antibody
GNE 适用: 人 WB, IHC 宿主: 兔 Polyclonal unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) antibodyGNE 适用: 人, 小鼠, 大鼠 WB, IHC, ELISA, IF, ICC 宿主: 兔 Polyclonal unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) (C-Term) antibodyGNE 适用: 人 WB, IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) antibodyGNE 适用: 人 WB 宿主: 兔 Monoclonal unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) (AA 1-110) antibodyGNE 适用: 人 WB, ELISA 宿主: 小鼠 Polyclonal unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) (C-Term) antibodyGNE 适用: 人, 小鼠, 大鼠 WB, IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) (AA 1-722) antibodyGNE 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) (AA 580-607), (C-Term) antibodyGNE 适用: 人, 小鼠 WB, FACS 宿主: 兔 Polyclonal RB26213 unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) (AA 169-197), (N-Term) antibodyGNE 适用: 人 WB, IHC (p) 宿主: 兔 Polyclonal RB31032 unconjugated
anti-Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE) (AA 168-197), (N-Term) antibodyGNE 适用: 人 WB, IHC (p), EIA 宿主: 兔 Polyclonal unconjugated
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应用备注
- IF(IHC-P) 1:50-200
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限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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有效期
- 12 months
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- GNE (Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE))
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别名
- GLCNE
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背景
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Synonyms: IBM2, Uae1, Bunctional 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_HUMAN.
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. Both IBM2 and NM/DMRV are autosomal recessive neuromuscular disorders characterized by adult onset, distal and proximal muscle weakness (especially in the legs) and a typical muscle pathology including filamentous inclusions and rimmed vacuoles.
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基因ID
- 10020
抗原
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