diphosphoric acid has been researched along with Pseudoxanthoma Elasticum in 15 studies
diphosphoric acid : An acyclic phosphorus acid anhydride obtained by condensation of two molecules of phosphoric acid.
Pseudoxanthoma Elasticum: An inherited disorder of connective tissue with extensive degeneration and calcification of ELASTIC TISSUE primarily in the skin, eye, and vasculature. At least two forms exist, autosomal recessive and autosomal dominant. This disorder is caused by mutations of one of the ATP-BINDING CASSETTE TRANSPORTERS. Patients are predisposed to MYOCARDIAL INFARCTION and GASTROINTESTINAL HEMORRHAGE.
Excerpt | Relevance | Reference |
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" These hereditary disorders, caused by mutations in genes encoding ATP binding cassette subfamily C member 6, ectonucleotide pyrophosphatase/phosphodiesterase 1, CD73, progressive ankylosis protein, and lamin A/C proteins, respectively, are inorganic pyrophosphate (PPi) deficiency syndromes with reduced circulating levels of PPi, the principal physiologic inhibitor of calcium hydroxyapatite deposition in soft connective tissues." | 9.22 | Inorganic Pyrophosphate Deficiency Syndromes and Potential Treatments for Pathologic Tissue Calcification. ( Li, Q; Ralph, D; Uitto, J; van de Wetering, K, 2022) |
" These hereditary disorders, caused by mutations in genes encoding ATP binding cassette subfamily C member 6, ectonucleotide pyrophosphatase/phosphodiesterase 1, CD73, progressive ankylosis protein, and lamin A/C proteins, respectively, are inorganic pyrophosphate (PPi) deficiency syndromes with reduced circulating levels of PPi, the principal physiologic inhibitor of calcium hydroxyapatite deposition in soft connective tissues." | 5.22 | Inorganic Pyrophosphate Deficiency Syndromes and Potential Treatments for Pathologic Tissue Calcification. ( Li, Q; Ralph, D; Uitto, J; van de Wetering, K, 2022) |
"Pseudoxanthoma elasticum is a prototype of heritable ectopic mineralization disorders, with phenotypic overlap with generalized arterial calcification of infancy and arterial calcification due to CD73 deficiency." | 2.55 | Insights into Pathomechanisms and Treatment Development in Heritable Ectopic Mineralization Disorders: Summary of the PXE International Biennial Research Symposium-2016. ( Li, Q; Terry, SF; Uitto, J; van de Wetering, K; Váradi, A, 2017) |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 0 (0.00) | 18.7374 |
1990's | 0 (0.00) | 18.2507 |
2000's | 0 (0.00) | 29.6817 |
2010's | 9 (60.00) | 24.3611 |
2020's | 6 (40.00) | 2.80 |
Authors | Studies |
---|---|
Kozák, E | 1 |
Fülöp, K | 1 |
Tőkési, N | 1 |
Rao, N | 1 |
Li, Q | 6 |
Terry, SF | 3 |
Uitto, J | 6 |
Zhang, X | 1 |
Becker, C | 1 |
Váradi, A | 5 |
Pomozi, V | 3 |
Ralph, D | 1 |
van de Wetering, K | 6 |
Murcia Casas, B | 1 |
Carrillo Linares, JL | 1 |
Baquero Aranda, I | 1 |
Rioja Villodres, J | 1 |
Merino Bohórquez, V | 1 |
González Jiménez, A | 1 |
Rico Corral, MÁ | 1 |
Bosch, R | 1 |
Sánchez Chaparro, MÁ | 1 |
García Fernández, M | 1 |
Valdivielso, P | 1 |
Letavernier, E | 1 |
Bouderlique, E | 1 |
Zaworski, J | 1 |
Martin, L | 3 |
Daudon, M | 1 |
Boyce, AM | 1 |
Gafni, RI | 1 |
Ferreira, CR | 1 |
Luo, H | 1 |
Faghankhani, M | 1 |
Cao, Y | 1 |
Shimada, BK | 1 |
Zoll, J | 2 |
Kuo, S | 1 |
Le Saux, O | 2 |
Brampton, C | 1 |
Calio, B | 1 |
Pham, K | 1 |
Owens, JB | 1 |
Marh, J | 1 |
Moisyadi, S | 1 |
Bauer, C | 1 |
Erdmann, J | 1 |
Aherrahrou, Z | 1 |
Germain, DP | 1 |
Jansen, RS | 2 |
Küçükosmanoglu, A | 1 |
de Haas, M | 1 |
Sapthu, S | 1 |
Otero, JA | 1 |
Hegman, IE | 1 |
Bergen, AA | 2 |
Gorgels, TG | 1 |
Borst, P | 2 |
Boraldi, F | 1 |
Annovi, G | 1 |
Bartolomeo, A | 1 |
Quaglino, D | 1 |
Duijst, S | 1 |
Mahakena, S | 1 |
Sommer, D | 1 |
Szeri, F | 1 |
Plomp, A | 1 |
Oude Elferink, RP | 1 |
Arányi, T | 1 |
Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
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Employment Retention Issues for Patient With Pseudoxanthoma Elasticum[NCT05246189] | 99 participants (Actual) | Observational | 2022-04-07 | Completed | |||
Intestinal Microbiota and Vitamin K Levels in PXE Patients (IMPROVE Study)[NCT03813550] | 20 participants (Anticipated) | Interventional | 2019-01-21 | Recruiting | |||
Response to Oral Lansoprazole of Inorganic Pyrophosphate Levels in Patients With Grönblad-Stranberg Disease (Pseudoxanthoma Elasticum)[NCT04660461] | Phase 4 | 20 participants (Anticipated) | Interventional | 2020-02-04 | Recruiting | ||
Biological Collection of Renal Cells for the Functional Study of the ABCC6 Transporter on iPS-derived Hepatocytes and Renal Cells[NCT03364504] | 10 participants (Anticipated) | Interventional | 2018-01-31 | Not yet recruiting | |||
[information is prepared from clinicaltrials.gov, extracted Sep-2024] |
9 reviews available for diphosphoric acid and Pseudoxanthoma Elasticum
Article | Year |
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Inorganic Pyrophosphate Deficiency Syndromes and Potential Treatments for Pathologic Tissue Calcification.
Topics: Ankylosis; Calcinosis; Choristoma; Diphosphates; Humans; Pseudoxanthoma Elasticum; Syndrome; Vascula | 2022 |
Pseudoxanthoma Elasticum, Kidney Stones and Pyrophosphate: From a Rare Disease to Urolithiasis and Vascular Calcifications.
Topics: 5'-Nucleotidase; Diphosphates; GPI-Linked Proteins; Humans; Kidney Calculi; Multidrug Resistance-Ass | 2019 |
Generalized Arterial Calcification of Infancy: New Insights, Controversies, and Approach to Management.
Topics: Adenosine Monophosphate; Bone Density Conservation Agents; Calcinosis; Cardiovascular Agents; Chelat | 2020 |
Molecular Genetics and Modifier Genes in Pseudoxanthoma Elasticum, a Heritable Multisystem Ectopic Mineralization Disorder.
Topics: Animals; Diphosphates; Genetic Association Studies; Humans; Mice; Multidrug Resistance-Associated Pr | 2021 |
ABCC6, Pyrophosphate and Ectopic Calcification: Therapeutic Solutions.
Topics: 5'-Nucleotidase; Animals; ATP-Binding Cassette Transporters; Calcification, Physiologic; Calcinosis; | 2021 |
Insights into Pathomechanisms and Treatment Development in Heritable Ectopic Mineralization Disorders: Summary of the PXE International Biennial Research Symposium-2016.
Topics: 5'-Nucleotidase; Alkaline Phosphatase; Animals; Biopsy, Needle; Clinical Trials as Topic; Congresses | 2017 |
Pseudoxanthoma elasticum.
Topics: Animals; Choroidal Neovascularization; Diphosphates; Humans; Metabolic Diseases; Peripheral Arterial | 2017 |
Pseudoxanthoma Elasticum as a Paradigm of Heritable Ectopic Mineralization Disorders: Pathomechanisms and Treatment Development.
Topics: 5'-Nucleotidase; Diphosphates; Diphosphonates; GPI-Linked Proteins; Humans; Multidrug Resistance-Ass | 2019 |
Research Progress in Pseudoxanthoma Elasticum and Related Ectopic Mineralization Disorders.
Topics: 5'-Nucleotidase; Animals; Biomedical Research; Diphosphates; Forecasting; Genetic Predisposition to | 2016 |
1 trial available for diphosphoric acid and Pseudoxanthoma Elasticum
Article | Year |
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Lansoprazole Increases Inorganic Pyrophosphate in Patients with Pseudoxanthoma Elasticum: A Double-Blind, Randomized, Placebo-Controlled Crossover Trial.
Topics: Cross-Over Studies; Diphosphates; Double-Blind Method; Humans; Phosphoric Diester Hydrolases; Pseudo | 2023 |
5 other studies available for diphosphoric acid and Pseudoxanthoma Elasticum
Article | Year |
---|---|
Oral supplementation of inorganic pyrophosphate in pseudoxanthoma elasticum.
Topics: Animals; Dietary Supplements; Diphosphates; Humans; Mice; Mutation; Phosphoric Diester Hydrolases; P | 2022 |
Pyrophosphate Supplementation Prevents Chronic and Acute Calcification in ABCC6-Deficient Mice.
Topics: Acute Disease; Animals; ATP-Binding Cassette Transporters; Calcinosis; Chronic Disease; Diphosphates | 2017 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
ABCC6 prevents ectopic mineralization seen in pseudoxanthoma elasticum by inducing cellular nucleotide release.
Topics: Animals; Dinucleoside Phosphates; Diphosphates; DNA Primers; DNA, Complementary; HEK293 Cells; Human | 2013 |
Fibroblasts from patients affected by Pseudoxanthoma elasticum exhibit an altered PPi metabolism and are more responsive to pro-calcifying stimuli.
Topics: Adult; Alkaline Phosphatase; Calcinosis; Calcium; Calcium-Binding Proteins; Diphosphates; DNA Primer | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |
ABCC6-mediated ATP secretion by the liver is the main source of the mineralization inhibitor inorganic pyrophosphate in the systemic circulation-brief report.
Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C | 2014 |