ArticleThe American journal of pathology2010
Parabiotic heterogenetic pairing of Abcc6-/-/Rag1-/- mice and their wild-type counterparts halts ectopic mineralization in a murine model of pseudoxanthoma elasticum.
Article in The American journal of pathology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 48 papers.
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Who cites it
48 citing papers in PubMed, 68 citations in OpenAlex.
- ABCC6 in Cellular Metabolic Homeostasis: Biochemical Links to Extracellular Nucleotide Signaling, Mitochondrial Stress, and Senescence.Cell biochemistry and biophysics · 2026Review
- Liver-directed base editing ofMolecular therapy. Nucleic acids · 2025Article
- ABCC6 Involvement in Cerebral Small Vessel Disease: Potential Mechanisms and Associations.Genes · 2025Review
- Managing Choroidal Neovascularization in Pseudoxanthoma Elasticum: Outcomes of Vitrectomy and Intravitreal Ranibizumab/Aflibercept Therapy-A Case Report.Case reports in ophthalmological medicine · 2025Article
- Pseudoxanthoma elasticum - Genetics, pathophysiology, and clinical presentation.Progress in retinal and eye research · 2024Review
- Angioid Streaks Remain a Challenge in Diagnosis, Management, and Treatment.Vision (Basel, Switzerland) · 2024Review
- The Purinergic Nature of Pseudoxanthoma Elasticum.Biology · 2024Review
- Review
- ABCC6, Pyrophosphate and Ectopic Calcification: Therapeutic Solutions.International journal of molecular sciences · 2021Review
- Cellular and Molecular Biomarkers Indicate Premature Aging in Pseudoxanthoma Elasticum Patients.Aging and disease · 2020Article
- Quantitative Trait Locus and Integrative Genomics Revealed Candidate Modifier Genes for Ectopic Mineralization in Mouse Models of Pseudoxanthoma Elasticum.The Journal of investigative dermatology · 2019Article
- Adenovirus-Mediated ABCC6 Gene Therapy for Heritable Ectopic Mineralization Disorders.The Journal of investigative dermatology · 2019Article
- Trauma-Induced Nanohydroxyapatite Deposition in Skeletal Muscle is Sufficient to Drive Heterotopic Ossification.Calcified tissue international · 2019Article
- PXE, a Mysterious Inborn Error Clarified.Trends in biochemical sciences · 2019Review
- Tissue-nonspecific alkaline phosphatase: a promising target for pseudoxanthoma elasticum therapy.Annals of translational medicine · 2017Article
- Plasma PPi Deficiency Is the Major, but Not the Exclusive, Cause of Ectopic Mineralization in an Abcc6The Journal of investigative dermatology · 2017Article
- Optical coherence tomography analysis of evolution of Bruch's membrane features in angioid streaks.Eye (London, England) · 2017Article
- The ABCC6 Transporter: A New Player in Biomineralization.International journal of molecular sciences · 2017Review
- Ectopic calcification in pseudoxanthoma elasticum responds to inhibition of tissue-nonspecific alkaline phosphatase.Science translational medicine · 2017Article
- Pseudoxanthoma elasticum.Orphanet journal of rare diseases · 2017Review
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
Pseudoxanthoma elasticum (PXE), a pleiotropic heritable disorder, is characterized by ectopic mineralization of the connective tissues. This disease is caused by mutations in the ABCC6 gene, which is expressed primarily in the baso-lateral surface of hepatocytes, and Abcc6(-/-) mice develop progressive mineralization mimicking human PXE. To investigate the hypothesis that PXE is a metabolic disorder, potentially caused by the absence of antimineralization factor(s) in circulation, we used parabiotic pairing, ie, surgical joining of two mice, to create a shared circulation between various Abcc6 genotypic mice. To prevent immune reaction between the parabiotic animals, all mice were bred to be Rag1(-/-). Shared circulation between the parabiotic animals was confirmed by Evans blue dye injection and by quantitative PCR of blood cell genotypes. Pairing of Abcc6(-/-) mice with their wild-type counterparts halted the connective tissue mineralization in the knockout mice. Homogenetic wild-type and heterozygous pairings serving as controls were phenotypically unaffected by parabiosis. Consequently, the observations on the parabiotic mice support the notion that PXE is a metabolic disease, potentially due to absence of systemic antimineralization factor(s). These observations suggest that reintroduction of the critical antimineralization factors into circulation could provide a potential treatment for this, currently intractable, disease.
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