|ENNP1 expression is in plasma cells and also in a number of non-lymphoid tissues, including distal convoluted tuble of the kidney, chondrocytes and epididymis.|
MOLECULAR FAMILY NAME: Belongs to the ENPP family.
ENPP1 is a single-pass type-2 glycoprotein. It contains a large extracellular domain, a transmembrane domain and a shortNH2 cytoplasmic domain. ENPP1 belong to the ecto-nucleotide pyrophosphatase/phosphodiesterase (ENPP). ENPP1 is comprised of two identical disulfide-bonded subunits.
POST-TRANSCRIPTIONAL MODIFICATION: No information
ENPP1 is N-glycosylated. Autophosphorylation is part of catalytic cycle of phosphodiesterase/pyrophosphatase activity.
|LIGANDS AND MOLECULES ASSOCIATED WITH ENPP1: No information,|
ENPP1 belong to the ecto-nucleotide pyrophosphatase/phosphodiesterase (ENPP). This protein has broad and specificity and cleaves a variety of substrates, including phosphodiester bonds of nucleotides and nucleotide sugars and pyrophosphate bonds of nucleotides and nucleotide sugars. This protein may function to hyrolyze nucleoside 5' triphosphates to their corresponding monophosphates and may hydrolyze diadenosine polyphosphates. ENPP1 may play a role in the regulation of pyrophosphate production, the regulation of the availability of nucleotide sugaras in the endoplasmic reticulum and Golgi and the regulation of purinergic signaling.
BIOCHEMICAL ACTIVITY: No information.
DISEASE RELEVANCE AND FUNCTION OF ENPP1 IN INTACT ANIMAL
Mutations of ENPP1 have been associated with 'idiopathic' infantile arterial calcification, ossification of the posteroir longitudinal ligament of the spine (OPLL). OPLL is a common form of human myelopathy with a prevalence of as much as 40% in a variety of ethnic groups. Defects are also the cause of idiopathic infantile arterial calcification of the internal elastic lamina of muscular arteries and stenosis due to myointimal proliferation.
and insulin resistance.
PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF ENPP1: No information.
SUBSTRATES: No information.
ENZYMES WHICH MODIFY ENPP1: No information.
Database accession numbers
June 25, 2008
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