ArticleActa neuropathologica2007
Neurolysosomal pathology in human prosaposin deficiency suggests essential neurotrophic function of prosaposin.
Article in Acta neuropathologica, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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20 citing papers in PubMed, 35 citations in OpenAlex.
- Contrasting Effects of Clozapine and Risperidone on Cholesterol Metabolism, Synaptic Proteins, and Transcriptional Regulation in Human LUHMES Neurons.Molecular neurobiology · 2025Article
- Stereo-seq of the prefrontal cortex in aging and Alzheimer's disease.Nature communications · 2025Article
- Prosaposin is a novel coenzyme Q10-binding protein.Journal of clinical biochemistry and nutrition · 2024Article
- Prosaposin PS18 reduces dopaminergic neurodegeneration in a 6-hydroxydopamine rat model of Parkinson's disease.Scientific reports · 2023Article
- Hypoxic Preconditioned Neural Stem Cell-Derived Extracellular Vesicles Contain Distinct Protein Cargo from Their Normal Counterparts.Current issues in molecular biology · 2023Article
- Loss of Lysosomal Proteins Progranulin and Prosaposin Associated with Increased Neurofibrillary Tangle Development in Alzheimer Disease.Journal of neuropathology and experimental neurology · 2021Article
- Microglial Progranulin: Involvement in Alzheimer's Disease and Neurodegenerative Diseases.Cells · 2019Review
- Progranulin Deficient Mice Develop Nephrogenic Diabetes Insipidus.Aging and disease · 2018Article
- Astroglia as a cellular target for neuroprotection and treatment of neuro-psychiatric disorders.Glia · 2017Review
- Lysosomal storage diseases.Translational science of rare diseases · 2017Review
- The protective role of prosaposin and its receptors in the nervous system.Brain research · 2014Review
- Two Faces of Cathepsin D: Physiological Guardian Angel and Pathological Demon.Biology and medicine (Aligarh) · 2014Article
- Article
- GPR37 and GPR37L1 are receptors for the neuroprotective and glioprotective factors prosaptide and prosaposin.Proceedings of the National Academy of Sciences of the United States of America · 2013Article
- Decrease in prosaposin in the Dystrophic mdx mouse brain.PloS one · 2013Article
- Identification and validation of novel cerebrospinal fluid biomarkers for staging early Alzheimer's disease.PloS one · 2011Article
- Pathology and current treatment of neurodegenerative sphingolipidoses.Neuromolecular medicine · 2010Review
- Regional expression of prosaposin in the wild-type and saposin D-deficient mouse brain detected by an anti-mouse prosaposin-specific antibody.Proceedings of the Japan Academy. Series B, Physical and biological sciences · 2009Article
- An association analysis of murine anxiety genes in humans implicates novel candidate genes for anxiety disorders.Biological psychiatry · 2008Article
- Combined saposin deficiency: A rare occurrence.Medical journal, Armed Forces IndiaArticle
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Authors and funding
3 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
A neuropathologic study of three cases of prosaposin (pSap) deficiency (ages at death 27, 89 and 119 days), carried out in the standard autopsy tissues, revealed a neurolysosomal pathology different from that in the non-neuronal cells. Non-neuronal storage is represented by massive lysosomal accumulation of glycosphingolipids (glucosyl-, galactosyl-, lactosyl-, globotriaosylceramides, sulphatide, and ceramide). The lysosomes in the central and peripheral neurons were distended by pleomorphic non-lipid aggregates lacking specific staining and autofluorescence. Lipid storage was borderline in case 1, and at a low level in the other cases. Neurolysosomal storage was associated with massive ubiquitination, which was absent in the non-neuronal cells and which did not display any immunohistochemical aggresomal properties. Confocal microscopy and cross-correlation function analyses revealed a positive correlation between the ubiquitin signal and the late endosomal/lysosomal markers. We suppose that the neuropathology most probably reflects excessive influx of non-lipid material (either in bulk or as individual molecules) into the neurolysosomes. The cortical neurons appeared to be uniquely vulnerable to pSap deficiency. Whereas in case 1 they populated the cortex, in cases 2 and 3 they had been replaced by dense populations of both phagocytic microglia and astrocytes. We suggest that this massive neuronal loss reflects a cortical neuronal survival crisis precipitated by the lack of pSap. The results of our study may extend the knowledge of the neurotrophic function of pSap, which should be considered essential for the survival and maintenance of human cortical neurons.
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