ArticleAngewandte Chemie (International ed. in English)2020
Pigmentation Chemistry and Radical-Based Collagen Degradation in Alkaptonuria and Osteoarthritic Cartilage.
Article in Angewandte Chemie (International ed. in English), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 52 citations in OpenAlex.
- Quantifying the Functional Gap in Alkaptonuria Through Machine Learning and Clinical Data Integration.Bioengineering (Basel, Switzerland) · 2026Article
- An anatomical investigation of alkaptonuria: Novel insights into ochronosis of cartilage and bone.Journal of anatomy · 2025Article
- Exploring the Osteoinductive Potential of Bacterial Pyomelanin Derived fromInternational journal of molecular sciences · 2024Article
- Joint replacement risk is markedly increased in alkaptonuria (AKU) in those with prior arthroplasty.Molecular genetics and metabolism reports · 2024Article
- Unveiling Charge-Pair Salt-Bridge Interaction Between GAGs and Collagen Protein in Cartilage: Atomic Evidence from DNP-Enhanced ssNMR at Natural Isotopic Abundance.Journal of the American Chemical Society · 2024Article
- Characterization of dielectric properties and their impact on MAS-DNP NMR applications.Journal of magnetic resonance (San Diego, Calif. : 1997) · 2024Article
- Review
- Ochronotic Chondropathy: A Case Report.Biomedicines · 2023Article
- PyrroTriPol: a semi-rigid trityl-nitroxide for high field dynamic nuclear polarization.Chemical science · 2023Article
- Clinical development innovation in rare diseases: overcoming barriers to successful delivery of a randomised clinical trial in alkaptonuria-a mini-review.Orphanet journal of rare diseases · 2023Review
- Revisiting Quantification of Phenylalanine/Tyrosine Flux in the Ochronotic Pathway during Long-Term Nitisinone Treatment of Alkaptonuria.Metabolites · 2022Article
- Article
- Association of alkaptonuria and low dose nitisinone therapy with cataract formation in a large cohort of patients.JIMD reports · 2022Article
- Metabolomic studies in the inborn error of metabolism alkaptonuria reveal new biotransformations in tyrosine metabolism.Genes & diseases · 2022Article
- Dynamic nuclear polarization-enhanced, double-quantum filteredJournal of magnetic resonance (San Diego, Calif. : 1997) · 2022Article
- Total knee arthroplasty and physical therapy for arthropathy in alkaptonuria: A 4-year follow-up case report.Frontiers in surgery · 2022Article
- Numerical recipes for faster MAS-DNP simulations.Journal of magnetic resonance (San Diego, Calif. : 1997) · 2021Article
- Lipid Anchoring Improves Lubrication and Wear Resistance of the Collagen I Matrix.Langmuir : the ACS journal of surfaces and colloids · 2021Article
- Vitiligo, alkaptonuria, and nitisinone-A report of three families and review of the literature.JIMD reports · 2021Article
- Development of an Effective Therapy for Alkaptonuria - Lessons for Osteoarthritis.Rheumatology and immunology research · 2021Review
Corrections and comments
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Authors and funding
9 authors at 5 institutions in 2 countries.
Funding
Abstract
Alkaptonuria (AKU) is a rare disease characterized by high levels of homogentisic acid (HGA); patients suffer from tissue ochronosis: dark brown pigmentation, especially of joint cartilage, leading to severe early osteoarthropathy. No molecular mechanism links elevated HGA to ochronosis; the pigment's chemical identity is still not known, nor how it induces joint cartilage degradation. Here we give key insight on HGA-derived pigment composition and collagen disruption in AKU cartilage. Synthetic pigment and pigmented human cartilage tissue both showed hydroquinone-resembling NMR signals. EPR spectroscopy showed that the synthetic pigment contains radicals. Moreover, we observed intrastrand disruption of collagen triple helix in pigmented AKU human cartilage, and in cartilage from patients with osteoarthritis. We propose that collagen degradation can occur via transient glycyl radicals, the formation of which is enhanced in AKU due to the redox environment generated by pigmentation.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.