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CTNS 抗体 (AA 231-330)

CTNS 适用: 人 ELISA, IHC (p), IF (cc), IF (p), IHC (fro), ICC 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1713755
发货至: 中国
  • 抗原 See all CTNS 抗体
    CTNS (Cystinosis, Nephropathic (CTNS))
    抗原表位
    • 8
    • 7
    • 6
    • 3
    • 2
    • 1
    • 1
    AA 231-330
    适用
    • 25
    • 5
    • 5
    • 1
    • 1
    • 1
    • 1
    • 1
    宿主
    • 22
    • 3
    克隆类型
    • 23
    • 2
    多克隆
    标记
    • 15
    • 2
    • 2
    • 2
    • 2
    • 2
    This CTNS antibody is un-conjugated
    应用范围
    ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunocytochemistry (ICC)
    预测反应
    Human,Mouse,Rat,Rabbit
    纯化方法
    Purified by Protein A.
    免疫原
    KLH conjugated synthetic peptide derived from human CTNS/Cystinosin
    亚型
    IgG
    Top Product
    Discover our top product CTNS Primary Antibody
  • 应用备注
    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
  • 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
    CTNS (Cystinosis, Nephropathic (CTNS))
    别名
    CTNS (CTNS 产品)
    别名
    CTNS-LSB antibody, PQLC4 antibody, AI195360 antibody, AW049661 antibody, zgc:110194 antibody, CTNS antibody, cystinosin, lysosomal cystine transporter antibody, cystinosis, nephropathic antibody, cystinosin, lysosomal cystine transporter L homeolog antibody, CTNS antibody, Ctns antibody, ctns antibody, ctns.L antibody
    背景

    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.

    基因ID
    1497
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