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Rabbit Anti-Lubricin/FITC Conjugated antibody
background:
Lubricin, also designated proteoglycan-4 or megakaryocyte stimulating factor, is important for boundary lubrication within articulating joints. It is a disulfide-linked homodimer (between Cysteine 1146 and Cysteine 1403) that is essential for protein cleavage. Lubricin inhibits synovial cell adhesion to the cartilage surface, but also prevents the deposition of proteins from synovial fluid onto cartilage. Lubricin is highly expressed in cartilage, liver and synovial tissue. Defects in the gene encoding for lubricin can cause Jakobs syndrome, also designated camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP). CACP is an autosomal recessive disorder characterized by joint failure associated with noninflammatory synoviocyte hyperplasia and subinitimal fibrosis of the synovial capsule. Lubricin undergoes different levels of glycosylation and may be detected at varying molecular weights.
Function:
Plays a role in boundary lubrication within articulating joints. Prevents protein deposition onto cartilage from synovial fluid by controlling adhesion-dependent synovial growth and inhibiting the adhesion of synovial cells to the cartilage surface.
Isoform F plays a role as a growth factor acting on the primitive cells of both hematopoietic and endothelial cell lineages.
soluble molecule that acts as a carrier for insoluble surface-active phospholipid (SAPL). Depletion of lubricin function has been associated with camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP), an arthritic-like autosomal recessive disorder.
Subunit:
Homodimer; disulfide-linked.
Subcellular Location:
Secreted.
Tissue Specificity:
Highly expressed in synovial tissue, cartilage and liver and weakly in heart and lung. Isoform B is expressed in kidney, lung, liver, heart and brain. Isoform C and isoform D are widely expressed.
Post-translational modifications:
N-glycosylated.
O-glycosylated; contains glycosaminoglycan chondroitin sulfate and keratan sulfate.
The disulfide bond between Cys-1146 and Cys-1403 is essential for protein cleavage.
DISEASE:
Defects in PRG4 are the cause of camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) [MIM:208250]; also known as Jacobs syndrome. CACP is an autosomal recessive disorder. Individuals with CACP have normal appearing joints at birth but with advancing age develop joint failure associated with noninflammatory synoviocyte hyperplasia and subintimal fibrosis of the synovial capsule.
Similarity:
Contains 2 hemopexin-like domains.
Contains 2 SMB (somatomedin-B) domains.
Database links:
Entrez Gene: 10216 Human
Entrez Gene: 280867 Cow
Entrez Gene: 96875 Mouse
Omim: 604283 Human
SwissProt: Q92954 Human
SwissProt: Q9JM99 Mouse
Unigene: 647723 Human
Unigene: 329131 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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