This gene encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes.
Its activity is driven by the H+ electrochemical gradient of the lysosomal membrane.
Mutations in this gene cause cystinosis, a lysosomal storage disorder.
Alternative splicing results in multiple transcript variants.
Applications:Suitable for use in Western Blot and ELISA.
Other applications not tested.
Recommended Dilutions:Optimal dilutions to be determined by the researcher.
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size:200ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by Protein A and peptide affinity chromatography.
Form:Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish Peroxidase (HRP).
Specificity:Recognizes human CTNS.
Isotype:IgG
Calc Applications Abbrev:E WB
Calc Crossreactivity:Hu
Immunogen:KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 341-367 in the C-terminal region of human CTNS, UniProt Accession #NP_004928.2; O60931.