Tumor Exosomal ENPP1 Hydrolyzes cGAMP to Inhibit cGAS‐STING Signaling
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DOI: 10.1002/advs.202308131
Abstract: To evade immune surveillance, tumor cells express ectonucleotide pyrophosphatase phosphodiesterase 1 (ENPP1) on the surface of their membrane, which degrades extracellular cyclic GMP‐AMP (cGAMP), thereby inhibiting the cyclic GMP‐AMP synthase (cGAS) stimulator of interferon gene… read more here.
Keywords: cgas; tumor; cgamp; exosomal enpp1 ... See more keywords
Response of the ENPP1‐Deficient Skeletal Phenotype to Oral Phosphate Supplementation and/or Enzyme Replacement Therapy: Comparative Studies in Humans and Mice
Sign Up to like & getrecommendations! Published in 2021 at "Journal of Bone and Mineral Research"
DOI: 10.1002/jbmr.4254
Abstract: Inactivating mutations in human ecto‐nucleotide pyrophosphatase/phosphodiesterase‐1 (ENPP1) may result in early‐onset osteoporosis (EOOP) in haploinsufficiency and autosomal recessive hypophosphatemic rickets (ARHR2) in homozygous deficiency. ARHR2 patients are frequently treated with phosphate supplementation to ameliorate the… read more here.
Keywords: enpp1; bone; bone mass; arhr2 patients ... See more keywords
Identification of ENPP1 Haploinsufficiency in Patients With Diffuse Idiopathic Skeletal Hyperostosis and Early‐Onset Osteoporosis
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Bone and Mineral Research"
DOI: 10.1002/jbmr.4550
Abstract: Homozygous ENPP1 mutations are associated with autosomal recessive hypophosphatemic rickets type 2 (ARHR2), severe ossification of the spinal ligaments, and generalized arterial calcification of infancy type 1. There are a limited number of reports on… read more here.
Keywords: enpp1; onset osteoporosis; early onset; case ... See more keywords
Differences in intracellular localisation of ANKH mutants that relate to mechanisms of calcium pyrophosphate deposition disease and craniometaphyseal dysplasia
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DOI: 10.1038/s41598-020-63911-x
Abstract: ANKH mutations are associated with calcium pyrophosphate deposition disease and craniometaphyseal dysplasia. This study investigated the effects of these ANKH mutants on cellular localisation and associated biochemistry. We generated four ANKH overexpression-plasmids containing either calcium pyrophosphate… read more here.
Keywords: pyrophosphate; ankh mutants; enpp1; localisation ... See more keywords
Identification of Downregulated Exosome-Associated Gene ENPP1 as a Novel Lipid Metabolism and Immune-Associated Biomarker for Hepatocellular Carcinoma
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Oncology"
DOI: 10.1155/2022/4834791
Abstract: Exosome plays an important role in the occurrence and development of tumors, such as hepatocellular carcinoma (LIHC). However, the functions and mechanisms of exosome-associated molecules in LIHC are still underexplored. Here, we investigated the role… read more here.
Keywords: enpp1; metabolism; hepatocellular carcinoma; exosome associated ... See more keywords
Abstract 577: ENPP1 inhibitor ZX-8177 in combination with chemotherapy or radiation exhibits synergistic anti-tumor effects via STING-mediated response
Sign Up to like & getrecommendations! Published in 2023 at "Cancer Research"
DOI: 10.1158/1538-7445.am2023-577
Abstract: 2’3’-cGAMP serves as a second messenger binding to the stimulator of interferon genes (STING) to induce the STING-dependent interferon response, triggering downstream antiviral and anti-tumor mechanism. Ecto-nucleotide pyrophosphatase/phosphodiesterase-1 (ENPP1) hydrolyzes the extracellular 2’3’-cGAMP and attenuates… read more here.
Keywords: enpp1; response; anti tumor; combination ... See more keywords
Abstract 488: Modulation of ENPP1 activity and 2'3'-cGAMP degradation in ovarian cancer cell lines via loadable pre-formed lipid nanoparticles
Sign Up to like & getrecommendations! Published in 2024 at "Cancer Research"
DOI: 10.1158/1538-7445.am2024-488
Abstract: Ovarian cancer is a complex heterogeneous disease with various subtypes and diverse molecular mechanisms of pathogenesis. Among different forms, epithelial ovarian cancer is the most common, representing up to 90% of total reported cases. Due… read more here.
Keywords: cancer; ovarian cancer; cgamp; cancer cell ... See more keywords
Tumor ENPP1(CD203a)/Haptoglobin Axis Exploits Myeloid-Derived Suppressor Cells to Promote Post-Radiotherapy Local Recurrence in Breast Cancer.
Sign Up to like & getrecommendations! Published in 2022 at "Cancer discovery"
DOI: 10.1158/2159-8290.cd-21-0932
Abstract: Locoregional failure (LRF) in breast cancer patients post-surgery and post-irradiation (IR) is linked to a dismal prognosis. In a refined new model, we identified Enpp1 (Ectonucleotide pyrophosphatase /phosphodiesterase 1/CD203a) to be closely associated with LRF.… read more here.
Keywords: post; cancer; breast cancer; tumor ... See more keywords
Correlation of baseline ENPP1 and cGAS expression in advanced solid tumors with intratumoral immune activation and clinical outcomes after treatment with the first-in-class oral ENPP1 inhibitor RBS2418, alone or in combination with pembrolizumab.
Sign Up to like & getrecommendations! Published in 2024 at "Journal of Clinical Oncology"
DOI: 10.1200/jco.2024.42.16_suppl.2597
Abstract: 2597 Background: ENPP1 is a nucleotide pyrophosphatase and phosphodiesterase. Its expression is associated with poor prognosis in cancer. ENPP1 inhibition protects cGAMP and ATP from hydrolysis and reduces adenosine levels in the TME, activates APCs… read more here.
Keywords: treatment; combination pembrolizumab; expression; immune activation ... See more keywords
Regulation of TGF-β1-induced fibroblast differentiation of human periodontal ligament stem cells through the mutually antagonistic action of ectonucleotide pyrophosphatase/phosphodiesterase 1 and 2
Sign Up to like & getrecommendations! Published in 2024 at "Frontiers in Cell and Developmental Biology"
DOI: 10.3389/fcell.2024.1426762
Abstract: Human periodontal ligament stem cells (hPDLSCs) differentiate into periodontal ligament (PDL) fibroblasts, osteoblasts, and cementoblasts. To identify inducers of PDL fibroblastic differentiation, monoclonal antibody series were developed a series of against membrane/extracellular matrix (ECM) molecules… read more here.
Keywords: differentiation; tgf induced; enpp1; periodontal ligament ... See more keywords
Case report: Multiple arterial stenoses induced by autosomal-recessive hypophosphatemic rickets type 2 associated with mutation of ENPP1: a case study
Sign Up to like & getrecommendations! Published in 2023 at "Frontiers in Cardiovascular Medicine"
DOI: 10.3389/fcvm.2023.1126445
Abstract: Ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1)-related multiple arterial stenoses is a rare clinical syndrome in which global arterial calcification begins in infancy, with a high probability of early mortality, and hypophosphatemic rickets develops later in childhood. The… read more here.
Keywords: enpp1; multiple arterial; arterial stenoses; case ... See more keywords