Results 91 to 100 of about 6,637 (212)

Enzyme Replacement Therapy With Elosulfase Alfa Decreases Storage of Glycosaminoglycan in White Blood Cells of Patients With Morquio A Syndrome

open access: yesJournal of Inborn Errors of Metabolism and Screening, 2015
Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is a lysosomal storage disorder caused by a deficient N-acetylgalactosamine-6-sulfate sulfatase activity, leading to cellular storage of undegraded keratan sulfate.
Guilherme Baldo PhD   +6 more
doaj   +1 more source

Macular corneal dystrophy with isolated peripheral Descemet membrane deposits

open access: yesAmerican Journal of Ophthalmology Case Reports, 2019
Purpose: Macular Corneal Dystrophy (MCD, MIM #217800) is a category 1 corneal stromal dystrophy as per the current IC3D classification. While characterized by macular stromal deposits, we report a case of MCD type II with isolated bilateral peripheral ...
Wenlin Zhang   +6 more
doaj   +1 more source

Distribution of proteoglycans antigenically related to corneal keratan sulfate proteoglycan

open access: yes, 1987
Three antibodies reacting with corneal keratan sulfate proteoglycan were used to detect antigenically related molecules in 11 bovine and 13 embryonic chick tissues. Two monoclonal antibodies recognized sulfated epitopes on the keratan sulfate chain and a
Caterson, B   +2 more
core  

Histamine H1-receptor-mediated keratan sulfate production in rabbit chondrocytes: involvement of protein kinase C

open access: yes, 1991
We investigated the characteristics of the histamine H1-receptor in cultured rabbit chondrocytes. Scatchard analysis of [3H]pyrilamine, an H1-antagonist, binding to the chondrocytes revealed a single class of binding sites with KD and Bmax values of 90 +/
T. Kuno   +6 more
core   +1 more source

Progressive Insights into 3D Bioprinting for Corneal Tissue Restoration

open access: yesAdvanced Healthcare Materials, Volume 15, Issue 3, 19 January 2026.
This review explores the potential of 3D bioprinting to replicate the complex structure and function of the human cornea. It highlights key advances in bioink development, printing modalities, and in vivo performance, while addressing current challenges and emerging strategies. The review emphasizes bioprinting's promise to overcome donor shortages and
Ilayda Namli   +6 more
wiley   +1 more source

Defective processing of keratan sulfate in Macular corneal dystrophy

open access: yes, 1984
macular corneal dystrophy is a human genetic disorder characterized by corneal opacities that arise, in part, from a failure to synthesize mature keratan sulfate proteoglycans.
Krachmer, J.   +5 more
core  

Monoclonal antibodies to rabbit corneal keratan sulfate proteoglycan

open access: yes, 1982
Six mouse hybrid cell lines were isolated which secrete antibodies to rabbit corneal proteoglycan. All six antibodies interacted with the same fraction of the proteoglycan, precipitating approximately 50% of proteoglycan labelled in the protein moiety. A
Funderburgh, James L.   +2 more
core   +1 more source

Synovial Fluid‐derived Micrococcus Luteus G18 Exacerbates Osteoarthritis Progression by Promoting Chondrocyte Degradation via TLR2/JNK/AP‐1 Signaling Pathway

open access: yesAdvanced Science, Volume 13, Issue 6, 30 January 2026.
Traditionally considered sterile, the articular cavity is now recognized to harbor microbial communities influencing osteoarthritis (OA). Integrating 16S rRNA sequencing and culturomics, Micrococcus luteus is enriched in advanced OA. Notably, M. luteus G18 exacerbates cartilage degeneration via TLR2/JNK/AP‐1‐mediated ECM disruption, revealing intra ...
Tingtao Chen   +6 more
wiley   +1 more source

From Molecule to Meaning: Neuronopathic Biomarkers and Clinical Relevance in GM1

open access: yesJournal of Inherited Metabolic Disease, Volume 49, Issue 1, January 2026.
ABSTRACT GM1 gangliosidosis is a rare, progressively neurodegenerative lysosomal storage disorder characterized by profound central nervous system involvement and substantial clinical heterogeneity. The development of reliable biomarkers is essential for tracking disease progression, stratifying patients, and advancing clinical trial readiness. Primary
Krista Casazza   +3 more
wiley   +1 more source

Cell surface-associated keratan sulfate on normal and migrating corneal endothelium.

open access: yes, 1996
To investigate cell surface-associated keratan sulfate on the corneal endothelium. METHODS. Immunolabeling techniques were used at the light, scanning, and transmission electron microscopic level to localize keratan sulfate on the corneal endothelium ...
Fullwood, Nigel J.   +5 more
core  

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