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CTNS Polyclonal Antibody

Applications

  • ELISA
  • IHC-P
  • IHC-F
  • IF(IHC-P)
  • IF(IHC-F)
  • IF(ICC)

Reactivity

  • Human
  • Mouse
  • Rat

Predicted Reactivity

  • Rabbit
Overview
Catalog # bs-12932R
Product Name CTNS Polyclonal Antibody
Applications ELISA, IHC-P, IHC-F, IF(IHC-P), IF(IHC-F), IF(ICC)
Reactivity Human, Mouse, Rat
Predicted Reactivity Rabbit
Specifications
Conjugation Unconjugated
Host Rabbit
Source KLH conjugated synthetic peptide derived from human CTNS/Cystinosin
Clonality Polyclonal
Clone # #REF!
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage Condition Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles.
Target
Gene ID 1497
Subcellular location Cytoplasm, Cell membrane
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.
Application Dilution
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