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GNE 抗体

GNE 适用: 人, 小鼠, 大鼠 WB, IF (p), IHC (p) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1385678
发货至: 中国
  • 抗原 See all GNE 抗体
    GNE (Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE))
    适用
    人, 小鼠, 大鼠
    宿主
    • 57
    • 1
    克隆类型
    • 57
    • 1
    多克隆
    标记
    • 18
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    This GNE antibody is un-conjugated
    应用范围
    • 52
    • 29
    • 18
    • 13
    • 12
    • 5
    • 1
    • 1
    • 1
    Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
    交叉反应
    人, 小鼠, 大鼠
    纯化方法
    Purified by Protein A.
    免疫原
    KLH conjugated synthetic peptide derived from human GLCNE
    亚型
    IgG
    Top Product
    Discover our top product GNE Primary Antibody
  • 应用备注
    WB 1:300-5000
    IHC-P 1:200-400
    IF(IHC-P) 1:50-200
    限制
    仅限研究用
  • 状态
    Liquid
    浓度
    1 μg/μL
    缓冲液
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
    储存液
    ProClin
    注意事项
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    储存条件
    4 °C,-20 °C
    储存方法
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    有效期
    12 months
  • 抗原
    GNE (Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE))
    别名
    GLCNE (GNE 产品)
    别名
    DMRV antibody, GLCNE antibody, IBM2 antibody, UAE1 antibody, zgc:77657 antibody, GNE antibody, MGC145505 antibody, NM antibody, Uae1 antibody, 2310066H07Rik antibody, glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase antibody, GNE antibody, gne antibody, Gne antibody
    背景

    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.

    基因ID
    10020
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