ArticleStructure (London, England : 1993)2014
A structure of a collagen VI VWA domain displays N and C termini at opposite sides of the protein.
Article in Structure (London, England : 1993), 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 1 of them a synthesis that pooled it.
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Who cites it
16 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Pooled it
- Genome-wide association identifies regulatory Loci associated with distinct local histogram emphysema patterns.American journal of respiratory and critical care medicine · 2014Trial
- Mutations in COL6A Gene Family Responsible for Muscular Dystrophies in Three Unrelated Families.Iranian biomedical journal · 2024Article
- Endotrophin, a Key Marker and Driver for Fibroinflammatory Disease.Endocrine reviews · 2024Review
- The coordinated activities of collagen VI and XII in maintenance of tissue structure, function and repair: evidence for a physical interaction.Frontiers in molecular biosciences · 2024Article
- Structural studies of elastic fibre and microfibrillar proteins.Matrix biology plus · 2021Article
- Protein Binder (ProBi) as a New Class of Structurally Robust Non-Antibody Protein Scaffold for Directed Evolution.Viruses · 2021Article
- Structure of a collagen VI α3 chain VWA domain array: adaptability and functional implications of myopathy causing mutations.The Journal of biological chemistry · 2020Article
- Structural and biophysical characterization of the type VII collagen vWFA2 subdomain leads to identification of two binding sites.FEBS open bio · 2020Article
- Structural and Biophysical Analysis of the CLCA1 VWA Domain Suggests Mode of TMEM16A Engagement.Cell reports · 2020Article
- CaComputational and structural biotechnology journal · 2020Article
- The pearl oyster Pinctada fucata martensii genome and multi-omic analyses provide insights into biomineralization.GigaScience · 2017Article
- Defining the hierarchical organisation of collagen VI microfibrils at nanometre to micrometre length scales.Acta biomaterialia · 2017Article
- Heterogeneity of Collagen VI Microfibrils: STRUCTURAL ANALYSIS OF NON-COLLAGENOUS REGIONS.The Journal of biological chemistry · 2016Article
- Architecture of the Human and Yeast General Transcription and DNA Repair Factor TFIIH.Molecular cell · 2015Article
- Characterization of recombinantly expressed matrilin VWA domains.Protein expression and purification · 2015Article
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5 authors.
Funding
Abstract
Von Willebrand factor A (VWA) domains are versatile protein interaction domains with N and C termini in close proximity placing spatial constraints on overall protein structure. The 1.2 Å crystal structures of a collagen VI VWA domain and a disease-causing point mutant show C-terminal extensions that place the N and C termini at opposite ends. This allows a "beads-on-a-string" arrangement of multiple VWA domains as observed for ten N-terminal domains of the collagen VI α3 chain. The extension is linked to the core domain by a salt bridge and two hydrophobic patches. Comparison of the wild-type and a muscular dystrophy-associated mutant structure identifies a potential perturbation of a protein interaction interface and indeed, the secretion of mutant collagen VI tetramers is affected. Homology modeling is used to locate a number of disease-associated mutations and analyze their structural impact, which will allow mechanistic analysis of collagen-VI-associated muscular dystrophy phenotypes.
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