ArticleHuman molecular genetics2008
Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice.
Article in Human molecular genetics, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 43 citations in OpenAlex.
- Accumulation of prosaposin and progranulin around the subfornical organ induces polydipsia in SAP-D-deficient mice.Biochemistry and biophysics reports · 2026Article
- Prosaposin Is Cleaved Into Saposins by Multiple Cathepsins in a Progranulin-Regulated Fashion.Journal of neurochemistry · 2026Article
- Experimental Analysis of Tear Fluid and Its Processing for the Diagnosis of Multiple Sclerosis.Sensors (Basel, Switzerland) · 2023Article
- Metachromatic Leukodystrophy: Diagnosis, Modeling, and Treatment Approaches.Frontiers in medicine · 2020Review
- The lipid transfer protein Saposin B does not directly bind CD1d for lipid antigen loading.Wellcome open research · 2019Article
- A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline.Neurobiology of disease · 2017Article
- Spiral ganglion degeneration and hearing loss as a consequence of satellite cell death in saposin B-deficient mice.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2015Article
- The protective role of prosaposin and its receptors in the nervous system.Brain research · 2014Review
- A novel homozygous splicing mutation in PSAP gene causes metachromatic leukodystrophy in two Moroccan brothers.Neurogenetics · 2014Article
- A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis.The Biochemical journal · 2013Article
- Inactivation of ceramide synthase 6 in mice results in an altered sphingolipid metabolism and behavioral abnormalities.The Journal of biological chemistry · 2013Article
- Gangliosides and gangliosidoses: principles of molecular and metabolic pathogenesis.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2013Review
- Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice.Human molecular genetics · 2013Article
- Chronic caloric restriction attenuates a loss of sulfatide content in PGC-1α-/- mouse cortex: a potential lipidomic role of PGC-1α in neurodegeneration.Journal of lipid research · 2012Article
- My journey into the world of sphingolipids and sphingolipidoses.Proceedings of the Japan Academy. Series B, Physical and biological sciences · 2012Review
- Ganglioside biochemistry.ISRN biochemistry · 2012Review
- Article
- Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models.Molecular genetics and metabolism · 2011Article
- Multi-system disorders of glycosphingolipid and ganglioside metabolism.Journal of lipid research · 2010Review
- The role of UDP-Glc:glycoprotein glucosyltransferase 1 in the maturation of an obligate substrate prosaposin.The Journal of cell biology · 2010Article
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
Abstract
Saposin B derives from the multi-functional precursor, prosaposin, and functions as an activity enhancer for several glycosphingolipid (GSL) hydrolases. Mutations in saposin B present in humans with phenotypes resembling metachromatic leukodystrophy. To gain insight into saposin B's physiological functions, a specific deficiency was created in mice by a knock-in mutation of an essential cysteine in exon 7 of the prosaposin locus. No saposin B protein was detected in the homozygotes (B-/-) mice, whereas prosaposin, and saposins A, C and D were at normal levels. B-/- mice exhibited slowly progressive neuromotor deterioration and minor head tremor by 15 months. Excess hydroxy and non-hydroxy fatty acid sulfatide levels were present in brain and kidney. Alcian blue positive (sulfatide) storage cells were found in the brain, spinal cord and kidney. Ultrastructural analyses showed lamellar inclusion material in the kidney, sciatic nerve, brain and spinal cord tissues. Lactosylceramide (LacCer) and globotriaosylceramide (TriCer) were increased in various tissues of B-/- mice supporting the in vivo role of saposin B in the degradation of these lipids. CD68 positive microglial cells and activated GFAP positive astrocytes showed a proinflammatory response in the brains of B-/- mice. These findings delineate the roles of saposin B for the in vivo degradation of several GSLs and its primary function in maintenance of CNS function. B-/- provide a useful model for understanding the contributions of this saposin to GSL metabolism and homeostasis.
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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.