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CTNS antibody (AA 231-330)

CTNS Reactivity: Human ELISA, IHC (p), ICC, IF (cc), IF (p), IHC (fro) Host: Rabbit Polyclonal unconjugated
Catalog No. ABIN1713755
  • Target See all CTNS Antibodies
    CTNS (Cystinosis, Nephropathic (CTNS))
    Binding Specificity
    • 8
    • 7
    • 6
    • 3
    • 2
    • 1
    • 1
    AA 231-330
    Reactivity
    • 25
    • 5
    • 5
    • 1
    • 1
    • 1
    • 1
    • 1
    Human
    Host
    • 22
    • 3
    Rabbit
    Clonality
    • 23
    • 2
    Polyclonal
    Conjugate
    • 15
    • 2
    • 2
    • 2
    • 2
    • 2
    This CTNS antibody is un-conjugated
    Application
    ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
    Predicted Reactivity
    Human,Mouse,Rat,Rabbit
    Purification
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human CTNS/Cystinosin
    Isotype
    IgG
    Top Product
    Discover our top product CTNS Primary Antibody
  • Application Notes
    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
    Restrictions
    For Research Use only
  • Format
    Liquid
    Concentration
    1 μg/μL
    Buffer
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
    Preservative
    ProClin
    Precaution of Use
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Storage
    4 °C,-20 °C
    Storage Comment
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    Expiry Date
    12 months
  • Target
    CTNS (Cystinosis, Nephropathic (CTNS))
    Alternative Name
    CTNS (CTNS Products)
    Synonyms
    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
    Background

    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.

    Gene ID
    1497
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