CTNS 抗体 (AA 231-330)
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北京 101111
Quick Overview for CTNS 抗体 (AA 231-330) (ABIN1713755)
抗原
See all CTNS 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 231-330
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预测反应
- Human,Mouse,Rat,Rabbit
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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 CTNS/Cystinosin
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亚型
- IgG
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anti-Cystinosis, Nephropathic (CTNS) (AA 1-100) antibody
CTNS 适用: 人 WB, ELISA 宿主: 小鼠 Monoclonal 5G6 unconjugated
anti-Cystinosis, Nephropathic (CTNS) (AA 1-400) antibodyCTNS 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-Cystinosis, Nephropathic (CTNS) (AA 341-367), (C-Term) antibodyCTNS 适用: 人 WB 宿主: 兔 Polyclonal RB40519 unconjugated
anti-Cystinosis, Nephropathic (CTNS) (AA 1-100) antibodyCTNS 适用: 人 WB, ELISA 宿主: 小鼠 Polyclonal unconjugated
anti-Cystinosis, Nephropathic (CTNS) (AA 1-100) antibodyCTNS 适用: 人, 小鼠, 大鼠 WB, ELISA, IHC, IHC (p) 宿主: 小鼠 Monoclonal 5G6 unconjugated
anti-Cystinosis, Nephropathic (CTNS) antibodyCTNS 适用: 人, 小鼠, 大鼠 IF 宿主: 兔 Polyclonal unconjugated
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应用备注
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ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500 -
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
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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
- 4 °C,-20 °C
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储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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有效期
- 12 months
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- CTNS (Cystinosis, Nephropathic (CTNS))
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别名
- CTNS
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背景
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Synonyms: CTNS LSB, Cystinosin, cystinosis, nephropathic, PQLC4, CTNS_HUMAN.
Background: Cystinosis is an autosomal recessive disorder resulting from defective lysosomal transport of cystine and present at birth as a failure to thrive, rickets and proximal renal tubular acidosis. The human CTNS gene on chromosome 17p13 encodes the protein Cystinosin, and mutations in CTNS are responsible for nephropathic cystinosis. The CTNS promoter contains an Sp1 binding element. Cystinosin is an integral membrane protein containing 7 transmembrane domains that functions as a H+-driven transporter responsible for cystine export from lysosomes. In humans, Cystinosin is expressed abundantly in pancreas, kidney (mature and fetal), and skeletal muscle. The mouse homolog to CTNS encodes a protein which is expressed in all tissues except skeletal muscle. In the cell, Cystinosin co-localizes with LAMP-2 to lysosomes. A C-terminal GYDQL sorting motif within Cystinosin is critical for lysosomal localization.
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基因ID
- 1497
抗原
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