SLC19A3 抗体 (C-Term)
Quick Overview for SLC19A3 抗体 (C-Term) (ABIN5516361)
抗原
See all SLC19A3 (Slc19a3) 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- C-Term
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序列
- VYGSYFAVIA GIFLMRSMYI TYSTKSQKDV QSPAPSENPD VSHPEEESNI
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纯化方法
- Affinity purified
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免疫原
- The immunogen is a synthetic peptide directed towards the C terminal region of human SLC19A3
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anti-Solute Carrier Family 19, Member 3 (Slc19a3) (Middle Region) antibody
Slc19a3 适用: 人, 小鼠, 大鼠, Cow, 犬, 豚鼠, 兔, 马 WB 宿主: 兔 Polyclonal unconjugated
anti-Solute Carrier Family 19, Member 3 (Slc19a3) (AA 191-282) antibodySlc19a3 适用: 人 WB, ELISA, IF 宿主: 兔 Polyclonal unconjugated
anti-Solute Carrier Family 19, Member 3 (Slc19a3) (AA 191-277) antibodySlc19a3 适用: 人 ELISA 宿主: 小鼠 Monoclonal 3B2 unconjugated
anti-Solute Carrier Family 19, Member 3 (Slc19a3) (AA 287-336) antibodySlc19a3 适用: 人, 小鼠, Cow, 犬, 豚鼠, 兔 WB 宿主: 兔 Polyclonal unconjugated
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应用备注
- Optimal working dilution should be determined by the investigator.
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: 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
- 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.
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- SLC19A3 (Slc19a3) (Solute Carrier Family 19, Member 3 (Slc19a3))
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别名
- SLC19A3
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背景
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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
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NCBI登录号
- NM_025243, NP_079519
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UniProt
- Q9BZV2
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途径
- Dicarboxylic Acid Transport
抗原
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