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Rabbit Anti-Lipoprotein lipase/FITC Conjugated antibody
background:
LPL encodes lipoprotein lipase, which is expressed in heart, muscle, and adipose tissue. LPL functions as a homodimer, and has the dual functions of triglyceride hydrolase and ligand/bridging factor for receptor-mediated lipoprotein uptake. Severe mutations that cause LPL deficiency result in type I hyperlipoproteinemia, while less extreme mutations in LPL are linked to many disorders of lipoprotein metabolism. [provided by RefSeq, Jul 2008]
Function:
The primary function of this lipase is the hydrolysis of triglycerides of circulating chylomicrons and very low density lipoproteins (VLDL). Binding to heparin sulfate proteogylcans at the cell surface is vital to the function. The apolipoprotein, APOC2, acts as a coactivator of LPL activity in the presence of lipids on the luminal surface of vascular endothelium.
Subunit:
Homodimer. Interacts with APOC2; the interaction activates LPL activity in the presence of lipids. Interacts with GPIHBP1.
Subcellular Location:
Cell membrane, Lipid-anchor, GPI-anchor. Secreted. Note=Locates to the plasma membrane of microvilli of hepatocytes with triacyl-glycerol-rich lipoproteins (TRL). Some of the bound LPL is then internalized and located inside non-coated endocytic vesicles.
Tissue Specificity:
Detected in intestinal microvilli, hair cell stereocilia, and fibroblast filopodia, in spleen and other lymph node-containing organs. Expressed in peripheral blood T lymphocytes, neutrophils, monocytes, B lymphocytes, and myeloid cells.
Post-translational modifications:
Tyrosine nitration after lipopolysaccharide (LPS) challenge down-regulates the lipase activity.
DISEASE:
Defects in LPL are the cause of lipoprotein lipase deficiency (LPL deficiency) [MIM:238600]; also known as familial chylomicronemia or hyperlipoproteinemia type I. LPL deficiency chylomicronemia is a recessive disorder usually manifesting in childhood. On a normal diet, patients often present with abdominal pain, hepatosplenomegaly, lipemia retinalis, eruptive xanthomata, and massive hypertriglyceridemia, sometimes complicated with acute pancreatitis.
Similarity:
Belongs to the AB hydrolase superfamily. Lipase family.
Contains 1 PLAT domain.
Database links:
Entrez Gene: 280843 Cow
Entrez Gene: 4023 Human
Entrez Gene: 16956 Mouse
Omim: 238600 Human
SwissProt: P11151 Cow
SwissProt: P06858 Human
SwissProt: P11152 Mouse
Unigene: 36878 Human
Unigene: 1514 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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