Page last updated: 2024-10-20

diphosphoric acid and Pseudoxanthoma Elasticum

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.

Research Excerpts

ExcerptRelevanceReference
" 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."9.22Inorganic 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."5.22Inorganic 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."2.55Insights 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)

Research

Studies (15)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's9 (60.00)24.3611
2020's6 (40.00)2.80

Authors

AuthorsStudies
Kozák, E1
Fülöp, K1
Tőkési, N1
Rao, N1
Li, Q6
Terry, SF3
Uitto, J6
Zhang, X1
Becker, C1
Váradi, A5
Pomozi, V3
Ralph, D1
van de Wetering, K6
Murcia Casas, B1
Carrillo Linares, JL1
Baquero Aranda, I1
Rioja Villodres, J1
Merino Bohórquez, V1
González Jiménez, A1
Rico Corral, MÁ1
Bosch, R1
Sánchez Chaparro, MÁ1
García Fernández, M1
Valdivielso, P1
Letavernier, E1
Bouderlique, E1
Zaworski, J1
Martin, L3
Daudon, M1
Boyce, AM1
Gafni, RI1
Ferreira, CR1
Luo, H1
Faghankhani, M1
Cao, Y1
Shimada, BK1
Zoll, J2
Kuo, S1
Le Saux, O2
Brampton, C1
Calio, B1
Pham, K1
Owens, JB1
Marh, J1
Moisyadi, S1
Bauer, C1
Erdmann, J1
Aherrahrou, Z1
Germain, DP1
Jansen, RS2
Küçükosmanoglu, A1
de Haas, M1
Sapthu, S1
Otero, JA1
Hegman, IE1
Bergen, AA2
Gorgels, TG1
Borst, P2
Boraldi, F1
Annovi, G1
Bartolomeo, A1
Quaglino, D1
Duijst, S1
Mahakena, S1
Sommer, D1
Szeri, F1
Plomp, A1
Oude Elferink, RP1
Arányi, T1

Clinical Trials (4)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Employment Retention Issues for Patient With Pseudoxanthoma Elasticum[NCT05246189]99 participants (Actual)Observational2022-04-07Completed
Intestinal Microbiota and Vitamin K Levels in PXE Patients (IMPROVE Study)[NCT03813550]20 participants (Anticipated)Interventional2019-01-21Recruiting
Response to Oral Lansoprazole of Inorganic Pyrophosphate Levels in Patients With Grönblad-Stranberg Disease (Pseudoxanthoma Elasticum)[NCT04660461]Phase 420 participants (Anticipated)Interventional2020-02-04Recruiting
Biological Collection of Renal Cells for the Functional Study of the ABCC6 Transporter on iPS-derived Hepatocytes and Renal Cells[NCT03364504]10 participants (Anticipated)Interventional2018-01-31Not yet recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

9 reviews available for diphosphoric acid and Pseudoxanthoma Elasticum

ArticleYear
Inorganic Pyrophosphate Deficiency Syndromes and Potential Treatments for Pathologic Tissue Calcification.
    The American journal of pathology, 2022, Volume: 192, Issue:5

    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.
    International journal of molecular sciences, 2019, Dec-17, Volume: 20, Issue:24

    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.
    Current osteoporosis reports, 2020, Volume: 18, Issue:3

    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.
    The Journal of investigative dermatology, 2021, Volume: 141, Issue:5

    Topics: Animals; Diphosphates; Genetic Association Studies; Humans; Mice; Multidrug Resistance-Associated Pr

2021
ABCC6, Pyrophosphate and Ectopic Calcification: Therapeutic Solutions.
    International journal of molecular sciences, 2021, Apr-27, Volume: 22, Issue:9

    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.
    The Journal of investigative dermatology, 2017, Volume: 137, Issue:4

    Topics: 5'-Nucleotidase; Alkaline Phosphatase; Animals; Biopsy, Needle; Clinical Trials as Topic; Congresses

2017
Pseudoxanthoma elasticum.
    Orphanet journal of rare diseases, 2017, 05-10, Volume: 12, Issue:1

    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.
    The American journal of pathology, 2019, Volume: 189, Issue:2

    Topics: 5'-Nucleotidase; Diphosphates; Diphosphonates; GPI-Linked Proteins; Humans; Multidrug Resistance-Ass

2019
Research Progress in Pseudoxanthoma Elasticum and Related Ectopic Mineralization Disorders.
    The Journal of investigative dermatology, 2016, Volume: 136, Issue:3

    Topics: 5'-Nucleotidase; Animals; Biomedical Research; Diphosphates; Forecasting; Genetic Predisposition to

2016

Trials

1 trial available for diphosphoric acid and Pseudoxanthoma Elasticum

ArticleYear
Lansoprazole Increases Inorganic Pyrophosphate in Patients with Pseudoxanthoma Elasticum: A Double-Blind, Randomized, Placebo-Controlled Crossover Trial.
    International journal of molecular sciences, 2023, Mar-03, Volume: 24, Issue:5

    Topics: Cross-Over Studies; Diphosphates; Double-Blind Method; Humans; Phosphoric Diester Hydrolases; Pseudo

2023

Other Studies

5 other studies available for diphosphoric acid and Pseudoxanthoma Elasticum

ArticleYear
Oral supplementation of inorganic pyrophosphate in pseudoxanthoma elasticum.
    Experimental dermatology, 2022, Volume: 31, Issue:4

    Topics: Animals; Dietary Supplements; Diphosphates; Humans; Mice; Mutation; Phosphoric Diester Hydrolases; P

2022
Pyrophosphate Supplementation Prevents Chronic and Acute Calcification in ABCC6-Deficient Mice.
    The American journal of pathology, 2017, Volume: 187, Issue:6

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Proceedings of the National Academy of Sciences of the United States of America, 2013, Dec-10, Volume: 110, Issue:50

    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.
    Journal of dermatological science, 2014, Volume: 74, Issue:1

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    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.
    Arteriosclerosis, thrombosis, and vascular biology, 2014, Volume: 34, Issue:9

    Topics: Adenosine Monophosphate; Adenosine Triphosphate; Aged; Animals; ATP-Binding Cassette Transporters; C

2014