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Rabbit Anti-TAP1/FITC Conjugated antibody
background:
The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is involved in the pumping of degraded cytosolic peptides across the endoplasmic reticulum into the membrane-bound compartment where class I molecules assemble. Mutations in this gene may be associated with ankylosing spondylitis, insulin-dependent diabetes mellitus, and celiac disease.
Function:
Involved in the transport of antigens from the cytoplasmto the endoplasmic reticulum for association with MHC class Imolecules. Also acts as a molecular scaffold for the final stage ofMHC class I folding, namely the binding of peptide. Nascent MHCclass I molecules associate with TAP via tapasin. Inhibited by thecovalent attachment of herpes simplex virus ICP47 protein, whichblocks the peptide-binding site of TAP. Inhibited by humancytomegalovirus US6 glycoprotein, which binds to the lumenal sideof the TAP complex and inhibits peptide translocation byspecifically blocking ATP-binding to TAP1 and prevents theconformational rearrangement of TAP induced by peptide binding.Inhibited by human adenovirus E3-19K glycoprotein, which binds theTAP complex and acts as a tapasin inhibitor, preventing MHC classI/TAP association. Expression of TAP1 is down-regulated by humanEpstein-Barr virus vIL-10 protein, thereby affecting the transportof peptides into the endoplasmic reticulum and subsequent peptideloading by MHC class I molecules.
Subunit:
Heterodimer of TAP1 and TAP2. Interacts with Epstein-Barrvirus BNLF2a. Interacts with PSMB5 and PSMB8.
Subcellular Location:
Endoplasmic reticulum membrane; Multi-passmembrane protein. Note=The transmembrane segments seem to form apore in the membrane.
DISEASE:
Bare lymphocyte syndrome 1 (BLS1) [MIM:604571]: A HLAclass I deficiency. Contrary to bare lymphocyte syndromes type 2and type 3, which are characterized by early-onset severe combinedimmunodeficiency, class I antigen deficiencies are not accompaniedby particular pathologic manifestations during the first years oflife. Systemic infections have not been described. Chronicbacterial infections, often beginning in the first decade of life,are restricted to the respiratory tract. Note=The disease is causedby mutations affecting the gene represented in this entry.
Similarity:
Belongs to the ABC transporter superfamily. ABCBfamily. MHC peptide exporter (TC 3.A.1.209) subfamily.
Contains 1 ABC transmembrane type-1 domain.
Contains 1 ABC transporter domain.
Database links:
Entrez Gene: 6890 Human
Entrez Gene: 24811 Rat
Omim: 170260 Human
SwissProt: Q03518 Human
SwissProt: P36370 Rat
Unigene: 352018 Human
Unigene: 10763 Rat
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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