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SLC19A3 抗体 (C-Term)

Slc19a3 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN5516361
发货至: 中国
  • 抗原 See all SLC19A3 (Slc19a3) 抗体
    SLC19A3 (Slc19a3) (Solute Carrier Family 19, Member 3 (Slc19a3))
    抗原表位
    • 7
    • 6
    • 6
    • 6
    • 4
    • 1
    • 1
    • 1
    • 1
    C-Term
    适用
    • 22
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    宿主
    • 20
    • 2
    克隆类型
    • 20
    • 2
    多克隆
    标记
    • 9
    • 3
    • 3
    • 3
    • 2
    • 2
    This SLC19A3 antibody is un-conjugated
    应用范围
    • 18
    • 17
    • 1
    • 1
    Western Blotting (WB)
    序列
    VYGSYFAVIA GIFLMRSMYI TYSTKSQKDV QSPAPSENPD VSHPEEESNI
    纯化方法
    Affinity purified
    免疫原
    The immunogen is a synthetic peptide directed towards the C terminal region of human SLC19A3
    Top Product
    Discover our top product Slc19a3 Primary Antibody
  • 应用备注
    Optimal working dilution should be determined by the investigator.
    限制
    仅限研究用
  • 状态
    Liquid
    缓冲液
    Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
    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
    -20 °C
    储存方法
    For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
  • 抗原
    SLC19A3 (Slc19a3) (Solute Carrier Family 19, Member 3 (Slc19a3))
    别名
    SLC19A3 (Slc19a3 产品)
    别名
    BBGD antibody, THMD2 antibody, THTR2 antibody, thtr2 antibody, MGC52872 antibody, MGC89434 antibody, si:dkey-223n17.4 antibody, slc19a3 antibody, A230084E24Rik antibody, AI788884 antibody, ThTr2 antibody, solute carrier family 19 member 3 antibody, solute carrier family 19 member 3 L homeolog antibody, solute carrier family 19 (thiamine transporter), member 3 antibody, thiamine transporter 2 antibody, solute carrier family 19 (thiamine transporter), member 3b antibody, solute carrier family 19, member 3 antibody, thiamine transporter 2-like antibody, SLC19A3 antibody, Slc19a3 antibody, slc19a3.L antibody, slc19a3 antibody, LOC486151 antibody, slc19a3b antibody, LOC100230080 antibody
    背景
    This gene encodes a ubiquitously expressed transmembrane thiamine transporter that lacks folate transport activity. Mutations in this gene cause biotin-responsive basal ganglia disease (BBGD), a recessive disorder manifested in childhood that progresses to chronic encephalopathy, dystonia, quadriparesis, and death if untreated. Patients with BBGD have bilateral necrosis in the head of the caudate nucleus and in the putamen. Administration of high doses of biotin in the early progression of the disorder eliminates pathological symptoms while delayed treatment results in residual paraparesis, mild mental retardation, or dystonia. Administration of thiamine is ineffective in the treatment of this disorder. Experiments have failed to show that this protein can transport biotin. Mutations in this gene also cause a Wernicke's-like encephalopathy.

    Alias Symbols: BBGD, THMD2, THTR2

    Protein Size: 496
    基因ID
    80704
    NCBI登录号
    NM_025243, NP_079519
    UniProt
    Q9BZV2
    途径
    Dicarboxylic Acid Transport
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