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Rabbit Anti-Tartrate Resistant Acid Phosphatase/FITC Conjugated antibody
background:
Tartrate-resistant acid phosphatase is an iron containing glycoprotein that catalyzes the conversion of orthophosphoric monoester to alcohol and orthophosphate. TRAP is the most basic of the acid phosphatases and is the only form not inhibited by L+-tartrate. TRAP is a relatively minor lysosomal enzyme which may be activated in certain pathologies such as Hodgkin’s disease and B- and T-cell leukemias. Receptor activator of NFκB ligand (RANKL) plays an essential role in osteoclast differentiation and activation by increasing the expression of protease osteoclast markers such as TRAP. TRAP has collagenolytic activity and plays a major role in ligament degradation.
Function:
Involved in osteopontin/bone sialoprotein dephosphorylation. Its expression seems to increase in certain pathological states such as Gaucher and Hodgkin diseases, the hairy cell, the B-cell, and the T-cell leukemias.
Subunit:
Exists either as monomer or, after proteolytic processing, as a dimer of two chains linked by disulfide bond(s).
Subcellular Location:
Lysosome.
DISEASE:
Defects in ACP5 are the cause of spondyloenchondrodysplasia with immune dysregulation (SPENCDI) [MIM:607944]. A disease characterized by vertebral and metaphyseal dysplasia, spasticity with cerebral calcifications, and strong predisposition to autoimmune diseases. The skeletal dysplasia is characterized by radiolucent and irregular spondylar and metaphyseal lesions that represent islands of chondroid tissue within bone. Note=ACP5 inactivating mutations result in a functional excess of phosphorylated osteopontin causing deregulation of osteopontin signaling and consequential autoimmune disease.
Similarity:
elongs to the metallophosphoesterase superfamily. Purple acid phosphatase family.
Database links:
Entrez Gene: 54 Human
Entrez Gene: 11433 Mouse
Entrez Gene: 25732 Rat
Omim: 171128 Human
SwissProt: P13686 Human
SwissProt: Q05117 Mouse
SwissProt: P29288 Rat
Unigene: 1211 Human
Unigene: 46354 Mouse
Unigene: 171928 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
抗酒石酸盐酸性磷酸酶(Tartrate-Resistant Acid Phosphatase,TRAP)是破骨细胞功能的重要标志物,它的活性与破骨细胞活性呈正相关.
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