ArticleNeuroscience2012
RPTPζ/phosphacan is abnormally glycosylated in a model of muscle-eye-brain disease lacking functional POMGnT1.
Article in Neuroscience, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 45 citations in OpenAlex.
- Contactin-1 is a critical neuronal cell surface receptor for perineuronal net structure.The Journal of biological chemistry · 2025Article
- Protein O-mannosylation: one sugar, several pathways, many functions.Glycobiology · 2023Review
- Involvement of abnormal dystroglycan expression and matriglycan levels in cancer pathogenesis.Cancer cell international · 2022Review
- On-tissue spatially resolved glycoproteomics guided by N-glycan imaging reveal global dysregulation of canine glioma glycoproteomic landscape.Cell chemical biology · 2022Article
- Retinal Proteomics of a Mouse Model of Dystroglycanopathies Reveals Molecular Alterations in Photoreceptors.Journal of proteome research · 2021Article
- Neural glycomics: the sweet side of nervous system functions.Cellular and molecular life sciences : CMLS · 2021Review
- The Expression, Functions, Interactions and Prognostic Values of PTPRZ1: A Review and Bioinformatic Analysis.Journal of Cancer · 2019Review
- Recent advancements in understanding mammalian O-mannosylation.Glycobiology · 2017Review
- MammalianThe Journal of biological chemistry · 2017Article
- Chondroitin sulfates and their binding molecules in the central nervous system.Glycoconjugate journal · 2017Review
- Neurodevelopmental Changes in Excitatory Synaptic Structure and Function in the Cerebral Cortex of Sanfilippo Syndrome IIIA Mice.Scientific reports · 2017Article
- Induction of Antibodies Directed Against Branched Core O-Mannosyl Glycopeptides-Selectivity Complimentary to the ConA Lectin.Chemistry (Weinheim an der Bergstrasse, Germany) · 2017Article
- A novel splice variant of the protein tyrosine phosphatase PTPRJ that encodes for a soluble protein involved in angiogenesis.Oncotarget · 2017Article
- Article
- Glycan susceptibility factors in autism spectrum disorders.Molecular aspects of medicine · 2016Review
- Protein O-Mannosylation in the Murine Brain: Occurrence of Mono-O-Mannosyl Glycans and Identification of New Substrates.PloS one · 2016Article
- Neurons and glia modify receptor protein-tyrosine phosphatase ζ (RPTPζ)/phosphacan with cell-specific O-mannosyl glycans in the developing brain.The Journal of biological chemistry · 2015Article
- Article
- Brevican knockdown reduces late-stage glioma tumor aggressiveness.Journal of neuro-oncology · 2014Article
- KSGal6ST is essential for the 6-sulfation of galactose within keratan sulfate in early postnatal brain.The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society · 2014Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Congenital muscular dystrophies (CMDs) with associated brain abnormalities are a group of disorders characterized by muscular dystrophy and brain and eye abnormalities that are frequently caused by mutations in known or putative glycotransferases involved in protein O-mannosyl glycosylation. Previous work identified α-dystroglycan as the major substrate for O-mannosylation and its altered glycosylation the major cause of these disorders. However, work from several labs indicated that other proteins in the brain are also O-mannosylated and therefore could contribute to CMD pathology in patients with mutations in the protein O-mannosylation pathway, however few of these proteins have been identified and fully characterized in CMDs. In this study we identify receptor protein tyrosine phosphatase ζ (RPTPζ) and its secreted variant, phosphacan, as another potentially important substrate for protein O-mannosylation in the brain. Using a mouse model of muscle-eye-brain disease lacking functional protein O-mannose β-1,2-N-acetylglucosaminyltransferase (POMGnT1), we show that RPTPζ/phosphacan is shifted to a lower molecular weight and distinct carbohydrate epitopes normally detected on the protein are either absent or substantially reduced, including Human Natural Killer-1 (HNK-1) reactivity. The spatial and temporal expression patterns of these O-mannosylated forms of RPTPζ/phosphacan and its hypoglycosylation and loss of HNK-1 glycan epitopes in POMGnT1 knockouts are suggestive of a role in the neural phenotypes observed in patients and animal models of CMDs.
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Registered trials
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.