免 疫 原：KLH conjugated synthetic peptide derived from human ENPP1
抗原表位：41-140/925
抗原细胞定位：Extracellular
SWISS：P22413
Gene ID ：5167
Human Gene ID：5167

ENPP1 has a broad specificity and cleaves a variety of substrates, including phosphodiester bonds of nucleotides and nucleotide sugars and pyrophosphate bonds of nucleotides and nucleotide sugars. It can hydrolyze nucleoside 5' triphosphates such as ATP, GTP, CTP, TTP and UTP to their corresponding monophosphates with release of pyrophosphate. It can also hydrolyze diadenosine polyphosphates and 3',5'-cAMP to AMP. It may play a role in the regulation of pyrophosphate production, the regulation of the availability of nucleotide sugars in the endoplasmic reticulum and Golgi, and the regulation of purinergic signaling.
Function：Involved primarily in ATP hydrolysis at the plasma membrane. Plays a role in regulating pyrophosphate levels, and functions in bone mineralization and soft tissue calcification. In vitro, has a broad specificity, hydrolyzing other nucleoside 5' triphospha
Subunit：In general heterodimer of an alpha and a beta chain linked by two disulfide bonds.
Subcellular Location：Membrane; Single-pass type II membrane protein. Basolateral cell membrane; Single-pass type II membrane protein. Note=Targeted to the basolateral membrane in polarized epithelial cells and in hepatocytes, and to matrix vesicles in osteoblasts. In bile duc
Tissue Specificity：Expressed in plasma cells and also in a number of non-lymphoid tissues, including the distal convoluted tubule of the kidney, chondrocytes and epididymis.
Post-translational modifications：Autophosphorylated as part of the catalytic cycle of phosphodiesterase/pyrophosphatase activity.
N-glycosylated.
It has been suggested that the active SMB domain may be permitted considerable disulfide bond heterogeneity or variability, thus two a
DISEASE：Defects in ENPP1 are a cause of increased susceptibility for ossification of the posterior longitudinal ligament of the spine (OPLL) [MIM:602475]. OPLL is a common form of human myelopathy with a prevalence of as much as 4% in a variety of ethnic groups.
Similarity：Belongs to the nucleotide pyrophosphatase/phosphodiesterase family.
Contains 2 SMB (somatomedin-B) domains.
Important Note：This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.