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Anti-CTNS Polyclonal Antibody is a Rabbit antibody targeting CTNS. Anti-CTNS Polyclonal Antibody can be used in WB.
| Pack Size | Price | USA Warehouse | Global Warehouse | Quantity |
|---|---|---|---|---|
| 50 μL | $222 | 7-10 days | 7-10 days | |
| 100 μL | $372 | 7-10 days | 7-10 days | |
| 200 μL | $527 | 7-10 days | 7-10 days |
| Description | Anti-CTNS Polyclonal Antibody is a Rabbit antibody targeting CTNS. Anti-CTNS Polyclonal Antibody can be used in WB. |
| Ig Type | IgG |
| Reactivity | Human,Mouse,Rat (predicted:Rabbit) |
| Application | |
| Recommended Dose | WB: 1:500-2000 |
| Antibody Type | Polyclonal |
| Host Species | Rabbit |
| Subcellular Localization | Lysosome membrane; Multi-pass membrane protein. |
| Tissue Specificity | Strongly expressed in pancreas, kidney (adult and fetal) and in skeletal muscle. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal). |
| Construction | Polyclonal Antibody |
| Purification | Protein A purified |
| Appearance | Liquid |
| Formulation | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Concentration | 1mg/ml |
| Research 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. |
| Immunogen | KLH conjugated synthetic peptide: human CTNS/Cystinosin |
| Antigen Species | Human |
| Gene Name | CTNS |
| Gene ID | |
| Protein Name | Cystinosin |
| Uniprot ID | |
| Function | CTNS (Cystinosin) is thought to transport cystine out of lysosomes. Mutations in the CTNS gene are the cause of cystinosis. |
| Molecular Weight | Theoretical: 42 kDa. |
| Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| Transport | Shipping with blue ice. |
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