Cystinosin Antibody (F-12): sc-100703

Cystinosin Antibody (F-12) is a mouse monoclonal IgG2a kappa light chain antibody that detects the Cystinosin protein of human origin by western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). Anti-Cystinosin antibody (F-12) is available as the non-conjugated form. Cystinosin plays a crucial role as an integral membrane protein with seven transmembrane domains, functioning as a H+-driven transporter responsible for the export of cystine from lysosomes. This function is vital because defective cystine transport leads to the accumulation of cystine within lysosomes, which is the underlying cause of cystinosis, an autosomal recessive disorder that manifests at birth with symptoms such as failure to thrive, rickets, and proximal renal tubular acidosis. The human CTNS gene, located on chromosome 17p13, encodes Cystinosin, and mutations in this gene are directly linked to nephropathic cystinosis. Notably, the CTNS promoter contains an Sp1 binding element, which is important for the regulation of gene expression. Cystinosin is predominantly expressed in the pancreas, kidney (both mature and fetal), and skeletal muscle, while its mouse homolog is found in all tissues except skeletal muscle. Within the cell, Cystinosin co-localizes with LAMP-2 in lysosomes, and a critical C-terminal GYDQL sorting motif is essential for proper lysosomal localization, underscoring the importance of structural integrity for function.

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Cystinosin Antibody (F-12) References:

1. Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter.  |  Kalatzis, V., et al. 2001. EMBO J. 20: 5940-9. PMID: 11689434
2. Cystinosin is a melanosomal protein that regulates melanin synthesis.  |  Chiaverini, C., et al. 2012. FASEB J. 26: 3779-89. PMID: 22649030
3. Cystinosin deficiency causes podocyte damage and loss associated with increased cell motility.  |  Ivanova, EA., et al. 2016. Kidney Int. 89: 1037-1048. PMID: 27083281
4. Cystinosis: a review.  |  Elmonem, MA., et al. 2016. Orphanet J Rare Dis. 11: 47. PMID: 27102039
5. Cystinosin-LKG rescues cystine accumulation and decreases apoptosis rate in cystinotic proximal tubular epithelial cells.  |  Taranta, A., et al. 2017. Pediatr Res. 81: 113-119. PMID: 27656773
6. The Role of Cystinosin in the Intermediary Thiol Metabolism and Redox Homeostasis in Kidney Proximal Tubular Cells.  |  Sumayao, R., et al. 2018. Antioxidants (Basel). 7: PMID: 30513914
7. Defective Cystinosin, Aberrant Autophagy-Endolysosome Pathways, and Storage Disease: Towards Assembling the Puzzle.  |  Rega, LR., et al. 2022. Cells. 11: PMID: 35159136
8. Structural basis for proton coupled cystine transport by cystinosin.  |  Löbel, M., et al. 2022. Nat Commun. 13: 4845. PMID: 35977944
9. Structure and mechanism of human cystine exporter cystinosin.  |  Guo, X., et al. 2022. Cell. 185: 3739-3752.e18. PMID: 36113465
10. Structural and mechanical insights into cystinosin.  |  Thomas, T. 2022. Nat Struct Mol Biol. 29: 955. PMID: 36220890
11. Ferroptosis inhibition by lysosome-dependent catabolism of extracellular protein.  |  Armenta, DA., et al. 2022. Cell Chem Biol. 29: 1588-1600.e7. PMID: 36306785
12. Evaluation of the efficacy of cystinosin supplementation through CTNS mRNA delivery in experimental models for cystinosis.  |  Bondue, T., et al. 2023. Sci Rep. 13: 20961. PMID: 38016974

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-Cystinosin Antibody (F-12) has 0 citations in a variety of scientific publications.

Primary antibodies like Anti-Cystinosin Antibody (F-12) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

Cystinosin Antibody (F-12) Specifications

Alternate Product Name: Cystinosin

Product Clone Name: F-12

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2a kappa light chain

Amino Acids: 1-101

Epitope Target: Cystinosin

Epitope Species: human

Product Reactivity/Detection: Cystinosin

Species Reactivity/Detection: human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, ELISA

Vialing Information: Each vial contains 100 µg IgG_2a kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 0 citations in various scientific publications

Available Conjugates/Forms for Cystinosin Antibody (F-12): N/A