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GCS1 抗体 (C-Term)

MOGS 适用: 人, 小鼠 WB 宿主: 兔 Polyclonal RB4963-4964 unconjugated
产品编号 ABIN389017
发货至: 中国
  • 抗原 See all GCS1 (MOGS) 抗体
    GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))
    抗原表位
    • 15
    • 7
    • 6
    • 5
    • 2
    • 1
    • 1
    AA 796-826, C-Term
    适用
    • 40
    • 13
    • 4
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    人, 小鼠
    宿主
    • 41
    克隆类型
    • 41
    多克隆
    标记
    • 17
    • 3
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This GCS1 antibody is un-conjugated
    应用范围
    • 41
    • 18
    • 13
    • 13
    • 3
    • 3
    • 3
    • 2
    • 2
    • 2
    • 1
    Western Blotting (WB)
    纯化方法
    This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
    免疫原
    This GCS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 796-826 amino acids from the C-terminal region of human GCS1.
    克隆位点
    RB4963-4964
    亚型
    Ig Fraction
    Top Product
    Discover our top product MOGS Primary Antibody
  • 应用备注
    WB: 1:1000
    限制
    仅限研究用
  • 状态
    Liquid
    缓冲液
    Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
    储存液
    Sodium azide
    注意事项
    This product contains Sodium azide: 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
    Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.
    有效期
    6 months
  • 抗原
    GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))
    别名
    GCS1 (MOGS 产品)
    别名
    Afu6g04210 antibody, AO090701000141 antibody, Mogs antibody, CDG2B antibody, CWH41 antibody, DER7 antibody, GCS1 antibody, 1810017N02Rik antibody, AI181835 antibody, Gcs1 antibody, gcs1 antibody, im:7160827 antibody, wu:fe50a12 antibody, wu:fk09a10 antibody, zgc:158312 antibody, mannosyl-oligosaccharide glucosidase antibody, mannosyl-oligosaccharide glucosidase GCS1 antibody, mannosyl-oligosaccharide glucosidase L homeolog antibody, mannosyl oligosaccharide glucosidase antibody, glucosidase 1 antibody, AFUA_6G04210 antibody, Tc00.1047053511015.10 antibody, Tc00.1047053511805.10 antibody, LOC5576381 antibody, AOR_1_260114 antibody, MGYG_00305 antibody, TERG_01248 antibody, mogs.L antibody, TTHERM_00636930 antibody, LOAG_03690 antibody, Gcs1 antibody, MOGS antibody, Mogs antibody, mogs antibody
    背景
    GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.
    分子量
    91918
    基因ID
    7841
    NCBI登录号
    NP_001139630, NP_006293
    UniProt
    Q13724
    途径
    SARS-CoV-2 Protein Interactome
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