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CTNS Antibody(C-term)

Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
  • WB - CTNS Antibody(C-term) AP19410b
    CTNS Antibody (C-term)(Cat. #AP19410b) western blot analysis in HepG2 cell line lysates (35ug/lane).This demonstrates the CTNS antibody detected the CTNS protein (arrow).
    detail
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
WB, E
Primary Accession O60931
Other Accession NP_004928.2
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 41738 Da
Antigen Region 341-367 aa
Additional Information
Gene ID 1497
Other Names Cystinosin, CTNS
Target/Specificity This CTNS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 341-367 amino acids from the C-terminal region of human CTNS.
Dilution WB~~1:1000
Format Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsCTNS Antibody(C-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name CTNS {ECO:0000303|PubMed:9537412, ECO:0000312|HGNC:HGNC:2518}
Function Cystine/H(+) symporter that mediates export of cystine, the oxidized dimer of cysteine, from lysosomes (PubMed:11689434, PubMed:18337546, PubMed:22232659, PubMed:29467429, PubMed:33208952, PubMed:15128704, PubMed:36113465). Plays an important role in melanin synthesis by catalyzing cystine export from melanosomes, possibly by inhibiting pheomelanin synthesis (PubMed:22649030). In addition to cystine export, also acts as a positive regulator of mTORC1 signaling in kidney proximal tubular cells, via interactions with components of the v-ATPase and Ragulator complexes (PubMed:36113465). Also involved in small GTPase-regulated vesicle trafficking and lysosomal localization of LAMP2A, independently of cystine transporter activity (By similarity).
Cellular Location [Isoform 1]: Lysosome membrane; Multi-pass membrane protein. Melanosome membrane; Multi-pass membrane protein. Note=AP-3 complex is required for localization to the lysosome.
Tissue Location Strongly expressed in pancreas, kidney (adult and fetal), skeletal muscle, melanocytes and keratinocytes (PubMed:22649030). Expressed at lower levels in placenta and heart Weakly expressed in lung, liver and brain (adult and fetal) (PubMed:22649030).
Research Areas
Citations (0)
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Background

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.

References

Taranta, A., et al. Pediatr. Nephrol. 25(7):1263-1267(2010)
Bellomo, F., et al. Free Radic. Biol. Med. 48(7):865-872(2010)
Kumar, A., et al. J. Biosci. 35(1):21-25(2010)
Aldahmesh, M.A., et al. Ophthalmic Genet. 30(4):185-189(2009)
Macias-Vidal, J., et al. Clin. Genet. 76(5):486-489(2009)

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$ 365.00
$ 140.00
Cat# AP19410b
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