ArticleJournal of cellular physiology2017
Reduced primary cilia length and altered Arl13b expression are associated with deregulated chondrocyte Hedgehog signaling in alkaptonuria.
Article in Journal of cellular physiology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 35 citations in OpenAlex.
- An in vitro cell model for exploring inflammatory and amyloidogenic events in alkaptonuria.Journal of cellular physiology · 2024Article
- Review
- Alkaptonuria.Nature reviews. Disease primers · 2024Review
- The role of a ciliary GTPase in the regulation of neuronal maturation of olfactory sensory neurons.Development (Cambridge, England) · 2023Article
- Recent advances in primary cilia in bone metabolism.Frontiers in endocrinology · 2023Review
- Article
- Ciliogenesis mechanisms mediated by PAK2-ARL13B signaling in brain endothelial cells is responsible for vascular stability.Biochemical pharmacology · 2022Article
- Cytoskeletal Protein 4.1G Is Essential for the Primary Ciliogenesis and Osteoblast Differentiation in Bone Formation.International journal of molecular sciences · 2022Article
- Role of Primary Cilia in Skeletal Disorders.Stem cells international · 2022Review
- Primary Cilia: A Cellular Regulator of Articular Cartilage Degeneration.Stem cells international · 2022Review
- Artificial Intelligence Approaches to Assessing Primary Cilia.Journal of visualized experiments : JoVE · 2021Article
- Estrogen withdrawal alters cytoskeletal and primary ciliary dynamics resulting in increased Hedgehog and osteoclastogenic paracrine signalling in osteocytes.Scientific reports · 2021Article
- Practical method for superresolution imaging of primary cilia and centrioles by expansion microscopy using an amplibody for fluorescence signal amplification.Molecular biology of the cell · 2020Article
- Article
- Ciliary IFT80 is essential for intervertebral disc development and maintenance.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2020Article
- Primary cilia: Versatile regulator in cartilage development.Cell proliferation · 2020Review
- Immunochromatographic assay to detect α-tubulin in urine for the diagnosis of kidney injury.Journal of clinical laboratory analysis · 2020Article
- Reduced primary cilia length and altered Arl13b expression are associated with deregulated chondrocyte Hedgehog signaling in alkaptonuria.Journal of cellular physiology · 2017Article
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Authors and funding
6 authors at 2 institutions in 2 countries.
Funding
Abstract
Alkaptonuria (AKU) is a rare inherited disease resulting from a deficiency of the enzyme homogentisate 1,2-dioxygenase which leads to the accumulation of homogentisic acid (HGA). AKU is characterized by severe cartilage degeneration, similar to that observed in osteoarthritis. Previous studies suggest that AKU is associated with alterations in cytoskeletal organization which could modulate primary cilia structure/function. This study investigated whether AKU is associated with changes in chondrocyte primary cilia and associated Hedgehog signaling which mediates cartilage degradation in osteoarthritis. Human articular chondrocytes were obtained from healthy and AKU donors. Additionally, healthy chondrocytes were treated with HGA to replicate AKU pathology (+HGA). Diseased cells exhibited shorter cilia with length reductions of 36% and 16% in AKU and +HGA chondrocytes respectively, when compared to healthy controls. Both AKU and +HGA chondrocytes demonstrated disruption of the usual cilia length regulation by actin contractility. Furthermore, the proportion of cilia with axoneme breaks and bulbous tips was increased in AKU chondrocytes consistent with defective regulation of ciliary trafficking. Distribution of the Hedgehog-related protein Arl13b along the ciliary axoneme was altered such that its localization was increased at the distal tip in AKU and +HGA chondrocytes. These changes in cilia structure/trafficking in AKU and +HGA chondrocytes were associated with a complete inability to activate Hedgehog signaling in response to exogenous ligand. Thus, we suggest that altered responsiveness to Hedgehog, as a consequence of cilia dysfunction, may be a contributing factor in the development of arthropathy highlighting the cilium as a novel target in AKU.
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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.