ArticleHuman molecular genetics2015
Ganglioside GM3 is essential for the structural integrity and function of cochlear hair cells.
Article in Human molecular genetics, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 57 citations in OpenAlex.
- Mapping ST3GAL transferase specificities in the glycosylation landscape of N/TERT-1 keratinocytes using Glycogenomics and mass spectrometry.Glycobiology · 2026Article
- Article
- When ganglioside pathways go awry: congenital disorders and experimental insights.Journal of human genetics · 2025Review
- Limited impact of cancer-derived gangliosides on anti-tumor immunity in colorectal cancer.Glycobiology · 2024Article
- The good, the bad, and the unknown nature of decreased GD3 synthase expression.Frontiers in molecular neuroscience · 2024Article
- Neural-specific alterations in glycosphingolipid biosynthesis and cell signaling associated with two human ganglioside GM3 synthase deficiency variants.Human molecular genetics · 2023Article
- Lipid rafts and human diseases: why we need to target gangliosides.FEBS open bio · 2023Review
- Article
- Neuronal Ganglioside and Glycosphingolipid (GSL) Metabolism and Disease : Cascades of Secondary Metabolic Errors Can Generate Complex Pathologies (in LSDs).Advances in neurobiology · 2023Article
- Functional Impairment of the Nervous System with Glycolipid Deficiencies.Advances in neurobiology · 2023Article
- Gangliosides in Neurodegenerative Diseases.Advances in neurobiology · 2023Article
- Ganglioside GM3 Synthase Deficiency in Mouse Models and Human Patients.International journal of molecular sciences · 2022Review
- Sex-Specific ADHD-like Behaviour, Altered Metabolic Functions, and Altered EEG Activity in Sialyltransferase ST3GAL5-Deficient Mice.Biomolecules · 2021Article
- Glycocalyx Curving the Membrane: Forces Emerging from the Cell Exterior.Annual review of cell and developmental biology · 2021Review
- A novel frameshift pathogenic variant in ST3GAL5 causing salt and pepper developmental regression syndrome (SPDRS): A case report.Human genome variation · 2021Article
- Roles of Gangliosides in Hypothalamic Control of Energy Balance: New Insights.International journal of molecular sciences · 2020Review
- Novel Molecular Mechanisms of Gangliosides in the Nervous System Elucidated by Genetic Engineering.International journal of molecular sciences · 2020Review
- Gangliosides in the Brain: Physiology, Pathophysiology and Therapeutic Applications.Frontiers in neuroscience · 2020Review
- Mass spectrometric quantification of plasma glycosphingolipids in human GM3 ganglioside deficiency.Clinical mass spectrometry (Del Mar, Calif.) · 2019Article
- Comparison of Human Tissue Microarray to Human Pericyte Transcriptome Yields Novel Perivascular Cell Markers.Stem cells and development · 2019Article
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Authors and funding
11 authors at 7 institutions in 2 countries.
Funding
Abstract
GM3 synthase (ST3GAL5) is the first biosynthetic enzyme of a- and b-series gangliosides. Patients with GM3 synthase deficiency suffer severe neurological disability and deafness. Eight children (ages 4.1 ± 2.3 years) homozygous for ST3GAL5 c.694C>T had no detectable GM3 (a-series) or GD3 (b-series) in plasma. Their auditory function was characterized by the absence of middle ear muscle reflexes, distortion product otoacoustic emissions and cochlear microphonics, as well as abnormal auditory brainstem responses and cortical auditory-evoked potentials. In St3gal5(-/-) mice, stereocilia of outer hair cells showed signs of degeneration as early as postnatal Day 3 (P3); thereafter, blebs devoid of actin or tubulin appeared at the region of vestigial kinocilia, suggesting impaired vesicular trafficking. Stereocilia of St3gal5(-/-) inner hair cells were fused by P17, and protein tyrosine phosphatase receptor Q, normally linked to myosin VI at the tapered base of stereocilia, was maldistributed along the cell membrane. B4galnt1(-/-) (GM2 synthase-deficient) mice expressing only GM3 and GD3 gangliosides had normal auditory structure and function. Thus, GM3-dependent membrane microdomains might be essential for the proper organization and maintenance of stereocilia in auditory hair cells.
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Registered trials
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