Phenotypic characterization of a Csf1r haploinsufficient mouse model of adult-onset leukodystrophy with axonal spheroids and pigmented glia (ALSP)
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Mutations in the colony stimulating factor-1 receptor (CSF1R) that abrogate the expression of the affected allele or lead to the expression of mutant receptor chains devoid of kinase activity have been identified in both familial and sporadic cases of ALSP. To determine the validity of the Csf1r heterozygous mouse as a model of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) we performed behavioral, radiologic, histopathologic, ultrastructural and cytokine expression studies of young and old Csf1r+/- and control Csf1r+/+ mice. Six to 8-month old Csf1r+/- mice exhibit cognitive deficits, and by 9-11 months develop sensorimotor deficits and in male mice, depression and anxiety-like behavior. MRIs of one year-old Csf1r+/- mice reveal lateral ventricle enlargement and thinning of the corpus callosum. Ultrastructural analysis of the corpus callosum uncovers dysmyelinated axons as well as neurodegeneration, evidenced by the presence of axonal spheroids. Histopathological examination of 11-week-old mice reveals increased axonal and myelin staining in the cortex, increase of neuronal cell density in layer V and increase of microglial cell densities throughout the brain, suggesting that early developmental changes contribute to disease. By 10-months of age, the neuronal cell density normalizes, oligodendrocyte precursor cells increase in layers II-III and V and microglial densities remain elevated without an increase in astrocytes. Also, the age-dependent increase in CSF-1R+ neurons in cortical layer V is reduced. Moreover, the expression of Csf2, Csf3, Il27 and Il6 family cytokines is increased, consistent with microglia-mediated inflammation. These results demonstrate that the inactivation of one Csf1r allele is sufficient to cause an ALSP-like disease in mice. The Csf1r+/- mouse is a model of ALSP that will allow the critical events for disease development to be determined and permit rapid evaluation of therapeutic approaches. Furthermore, our results suggest that aberrant activation of microglia in Csf1r+/- mice may play a central role in ALSP pathology.
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Recent work in a mouse Csf1r+/− model illustrated a potential role in neuronal development and hypermyelination in early disease.
“Recent work in a mouse Csf1r+/− model illustrated a potential role in neuronal development and hypermyelination in early disease [9].”
From Loss of homeostatic microglial phenotype in CSF1R-related Leukoencephalopathy (2020) · cites this paper as [9]
Targeting CSF1R has therapeutic potential, but caution is warranted because CSF1R haploinsufficiency is linked to adult-onset leukoencephalopathy with axonal spheroids and pigmented glia.
“Although targeting CSF1R has therapeutic potential, caution should be taken, as CSF1R haploinsufficiency was linked to adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (145).”
From Microglia in Alzheimer's disease (2017) · cites this paper as [145]
CSF1R haploinsufficiency has been implicated in the pathogenesis of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia.
“CSF1R haploinsufficiency has been implicated in the pathogenesis of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (193).”
From Microglia Function in the Central Nervous System During Health and Neurodegeneration (2017) · cites this paper as [193]
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“In a study of the Csf1r haploinsufficient mouse model of ALSP, depression and anxiety-like behaviour were observed only in male mice [41], but this was not seen in our study.”
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- Authors
- Chitu, V., Gokhan, S., Gulinello, M., Branch, C. A., Patil, M., Basu, R., Stoddart, C., Mehler, M. F., & Stanley, E. R.
- Affiliations
- Albert Einstein College of Medicine (US)
- Corresponding author
- Stanley, E. Richard
- Venue
- Neurobiology of Disease
- Publisher
- Elsevier BV
- Published
- 2015-02
- Volume
- 74
- Pages
- 219-228
- ISSN
- 0969-9961 (ISSN-L)
- PMC
- PMC4323933
- OpenAlex
- W2073301230
- Cited by
- 119
- References
- 61
- Citations by year
- 2026: 8 · 2025: 10 · 2024: 20 · 2023: 11 · 2022: 10 · 2021: 18 · 2020: 11 · 2019: 5 · 2018: 7 · 2017: 6 · 2016: 7 · 2015: 6
- FWCI
- 3.29
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Neurobiology of Disease (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Institutes of Health (CA32551, NS071571)
- Rose F. Kennedy Intellectual Disabilities Research Center (P30HD071593)
- Topics
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
- Life Sciences › Neuroscience › Developmental Neuroscience › Neurogenesis and neuroplasticity mechanisms
- Keywords
- adult-onset leukoencephalopathy with axonal spheroids and pigmented glia, CSF1R, haploinsufficiency, axonal swellings, dysmyelination, corpus callosum thinning, microglial activation, neuroinflammation, cognitive impairment, sensorimotor deficits, oligodendrocyte precursor cells, cytokine expression, neurodegeneration
- MeSH terms
- Animals, Anxiety, Brain, Cognition Disorders, Depression, Disease Models, Animal, Female, Male, Mice, Inbred C57BL, Motor Activity, Neuroglia, Neurons, Phenotype, Receptors, Granulocyte-Macrophage Colony-Stimulating Factor, Cytokines, Mice, Knockout, Disease Progression, Olfactory Perception, Leukoencephalopathies, Neural Stem Cells, White Matter
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