Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Enhanced lysosomal exocytosis and altered growth factor signaling are associated with cartilage pathology in a model of mucopolysaccharidosis type IVA.

Disease models & mechanisms - 1 Feb 2026

Lee Jen-Jie, Lu Po-Nien, Dukes-Rimsky Lynn, Jeter Chelsi, Colonna Maxwell B, Poplawski Andrzej B, Arno Gavin, Hallman Jenna, Underwood Christina, Basu Amrita, Pollard Laura, Weiss Ryan J, Steet Richard, Flanagan-Steet Heather

Abstract excerpt

Optimal lysosomal function is essential for early tissue development. This is evidenced by the large number of inherited disorders, collectively called the lysosomal storage disorders (LSDs), caused by lysosomal dysfunction. Although it is clear that macromolecular accumulation adversely impacts tissue development, the breadth of downstream pathways contributing to pathology has yet to be elucidated. Multiple...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Bone Morphogenetic Proteins
Share to Cartilage

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Cathepsins

Share to Exocytosis

Share to Glycosaminoglycans

Share to Lysosomes

Share to Transforming Growth Factor beta

Share to Zebrafish Proteins

Source

Open in PubMed Open full text

Identifiers

PMID: 41582713
DOI: 10.1242/dmm.052582
PMCID: PMC12994454

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Enhanced lysosomal exocytosis and altered growth factor signaling are associated with cartilage pathology in a model of mucopolysaccharidosis type IVA.PMID 41582713Read ↗

Cathepsin-Mediated Alterations in TGFß-Related Signaling Underlie Disrupted Cartilage and Bone Maturation Associated With Impaired Lysosomal Targeting.Semantic proximity Read ↗

The pathogenesis of lysosomal storage disorders: beyond the engorgement of lysosomes to abnormal development and neuroinflammationSemantic proximity Read ↗

Altered chondrocyte differentiation and extracellular matrix homeostasis in a zebrafish model for mucolipidosis II.Semantic proximity Read ↗

An Engineered sgsh Mutant Zebrafish Recapitulates Molecular and Behavioural Pathobiology of Sanfilippo Syndrome A/MPS IIIA.Semantic proximity Read ↗

FGF signaling deregulation is associated with early developmental skeletal defects in animal models for mucopolysaccharidosis type II (MPSII)Semantic proximity Read ↗

Cathepsins in the Pathophysiology of Mucopolysaccharidoses: New Perspectives for Therapy.Semantic proximity Read ↗

Inappropriate cathepsin K secretion promotes its enzymatic activation driving heart and valve malformation.Semantic proximity Read ↗

A single heterozygous mutation in <i>COG4</i> disrupts zebrafish early development via Wnt signalingSemantic proximity Read ↗

<i>b3galt6</i> knock-out zebrafish recapitulate β3GalT6-deficiency disorders in human and reveal a trisaccharide proteoglycan linkage regionSemantic proximity Read ↗

Defective collagen proteostasis and matrix formation in the pathogenesis of lysosomal storage disorders.Semantic proximity Read ↗

The Lysosomal Sialic Acid Transporter Sialin Is Required for Normal CNS MyelinationSemantic proximity Read ↗

Zebrafish models of Mucopolysaccharidosis IIIA, IIIB, and IIIC show hyperactivity, neuroinflammation and changes in oligodendrocyte cell stateSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.