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Rabbit Anti-Claudin 14/FITC Conjugated antibody
background:
The Claudin superfamily consists of many structurally related proteins in humans. These proteins are important structural and functional components of tight junctions in paracellular transport. Claudins are located in both epithelial and endothelial cells in all tight junction-bearing tissues. Three classes of proteins are known to localize to tight junctions, including the claudins, Occludin and Junction adhesion molecules. Claudins, which consist of four transmembrane domains and two extracellular loops, make up tight junction strands. Claudin expression is often highly restricted to specfic regions of different tissues and may have an important role in transcellular transport through tight junctions. Claudin-14 is a multi-pass membrane protein that is expressed in liver, kidney and ear. Defects in the gene encoding claudin-14 are the cause of non-syndromic sensorineural deafness autosomal recessive type 29 (DFNB29), a form of hearing loss resulting from damage to either nerve pathways or neural receptors of the inner ear.
Function:
Plays a major role in tight junction-specific obliteration of the intercellular space, through calcium-independent cell-adhesion activity. Acts as a co-receptor for HCV entry into hepatic cells.
Subunit:
Can form homo- and heteropolymers with other CLDN. Homopolymers interact with CLDN3, but not CLDN2, homopolymers. Directly interacts with TJP1/ZO-1, TJP2/ZO-2 and TJP3/ZO-3. Interacts with MPDZ and INADL (By similarity). May interact with HCV E1 and E2 proteins.
Subcellular Location:
Cell junction, tight junction. Cell membrane; Multi-pass membrane protein.
Tissue Specificity:
Strongly expressed in liver and kidney. Expressed in heart, brain, spleen, lung and testis.
DISEASE:
Defects in CLDN1 are the cause of ichthyosis-sclerosing cholangitis neonatal syndrome (NISCH) [MIM:607626]; also called ichthyosis with leukocyte vacuoles alopecia and sclerosing cholangitis (ILVASC). NISCH is a rare autosomal recessive complex ichthyosis syndrome characterized by scalp hypotrichosis, scarring alopecia, vulgar type ichthyosis, and sclerosing cholangitis.
Similarity:
Belongs to the claudin family.
Database links:
Entrez Gene: 23562 Human
Entrez Gene: 56173 Mouse
Entrez Gene: 304073 Rat
Omim: 605608 Human
SwissProt: O95500 Human
SwissProt: Q9Z0S3 Mouse
Unigene: 660278 Human
Unigene: 328716 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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