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Keratan sulfate (KS) is a glycosaminoglycan (GAG) with unique functions including electroconductive properties that support neurotransmission. KS-proteoglycans contribute to tissue stabilization and functional organization, and have diverse interactive properties with cytokines, growth factors, morphogens, neuregulatory proteins, and neuron receptors that control the formation and function of neural networks. As side chain components of a diverse range of brain proteoglycans, KS assists neural development and axonal guidance, storage and transport of neurotransmitters in synaptic vesicles, neurotransduction, synaptic plasticity, cognition, and memory in perineuronal nets, neuronal proliferation, and differentiation. KS is thus a multifunctional instructive brain GAG with essential roles to play in brain function and homeostasis, with instructive roles in the assembly and repair of functional network structures from secreted molecules produced by glial cells and neurons in the assembly of transmitter and effector receptors and ion channels which affect brain function and neuronal control. KS is a component of neuropils in white matter; however, its specific roles in the function of this tissue have yet to be determined. Dysfunctional KS-mediated cell signaling, however, may predispose to the development of a number of neurological disorders.
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http://dx.doi.org/10.1111/jnc.70208 | DOI Listing |
J Neurochem
August 2025
Raymond Purves Bone and Joint Research Laboratories, Kolling Institute of Medical Research, Faculty of Medicine and Health, The University of Sydney and the Northern Sydney Local Health District, Royal North Shore Hospital, Sydney, New South Wales, Australia.
Keratan sulfate (KS) is a glycosaminoglycan (GAG) with unique functions including electroconductive properties that support neurotransmission. KS-proteoglycans contribute to tissue stabilization and functional organization, and have diverse interactive properties with cytokines, growth factors, morphogens, neuregulatory proteins, and neuron receptors that control the formation and function of neural networks. As side chain components of a diverse range of brain proteoglycans, KS assists neural development and axonal guidance, storage and transport of neurotransmitters in synaptic vesicles, neurotransduction, synaptic plasticity, cognition, and memory in perineuronal nets, neuronal proliferation, and differentiation.
View Article and Find Full Text PDFJ Tradit Chin Med
August 2025
Department of Chinese Medicine, Hunan University of Chinese Medicine, Changsha 410208, China.
Objective: To utilize the Traditional Chinese Medicine constitution (TCMC) as a complementary and alternative approach for early disease detection and treatment, with a focus on and deficiency constitutions, which serve as key references for disease prevention and management.
Methods: The dataset containing the data of and deficiency constitution was identified through the Gene Expression Omnibus database. This database was used for differential expression genes (DEGs) analysis and weighted gene co-expression network analysis (WGCNA), and the characteristic genes were then obtained in the dataset using a machine learning method.
Am J Ophthalmol
August 2025
Stein Eye Institute, UCLA, Los Angeles CA. Electronic address:
Objective: To report a CHST6-associated corneal endothelial dystrophy.
Design: Prospective observational case series.
Participants: Thirty-five individuals from seven families, including 13 affected individuals exhibiting corneal epithelial and stromal edema, peripheral posterior corneal macular opacities, and endothelial guttae, as well as 22 unaffected family members.
Int J Mol Sci
July 2025
Nemours Children's Health, Wilmington, DE 19803, USA.
Mucopolysaccharidosis IVA (MPS IVA) is a lysosomal storage disorder causing systemic skeletal dysplasia due to a deficiency of N-acetyl-galactosamine-6-sulfate sulfatase (GALNS) enzyme activity, leading to the impaired degradation and accumulation of glycosaminoglycans (GAGs), keratan sulfate (KS) and chondroitin-6-sulfate. While treatments such as enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT) are available, they have significant limitations regarding efficacy in skeletal tissues and long-term safety, highlighting the need for more effective therapies. We evaluated a novel gene therapy approach using a dual Integrase-deficient lentiviral vector (IDLV) to deliver an expression cassette that includes human cDNA and Cas9 sgRNA, targeting the upstream region of the mouse initial codon.
View Article and Find Full Text PDFMol Ther Methods Clin Dev
September 2025
Department of Biological Sciences, University of Delaware, Newark, DE 19716, USA.
Mucopolysaccharidosis IVA (MPS IVA) is an autosomal recessive disorder that causes the accumulation of keratan sulfate (KS) and chondroitin-6-sulfate in bone and cartilage. This results in progressive skeletal dysplasia, with no effective treatment available. Our study hypothesized that direct lentiviral vector (LV) gene therapy could produce active enzymes from transduced cells, impacting bone and cartilage lesions in MPS IVA.
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