Cerebro-hepato-renal syndrome of Zellweger: an inherited disorder of neuronal migration
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In patients with Zellweger syndrome, the most prominent feature of brain pathology is malformation of the cortex, attributed to neuronal migration defects.
“In patients with Zellweger syndrome, the most prominent feature of the brain pathology is a malformation of the cortex, which has been attributed to neuronal migration defects.”
From Peroxisomes in brain development and function (2016) · cites this paper as [49]
The abnormalities in cytoarchitecture of the cerebral cortex in Zellweger syndrome are usually bilateral and approximately symmetrical.
“The abnormalities in the cytoarchitecture of the cerebral cortex are usually bilateral and approximately symmetrical [49,50].”
From Peroxisomes in brain development and function (2016) · cites this paper as [49]
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“The abnormalities in the cytoarchitecture of the cerebral cortex are usually bilateral and approximately symmetrical [49,50].”
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- Authors
- Volpe, J. J., & Adams, R. D.
- Affiliations
- St. Louis Children's Hospital (US)
- Harvard University (US)
- ORCID
- Adams, Raymond D.
- Venue
- Acta Neuropathologica
- Published
- 1972
- Volume
- 20
- Issue
- 3
- Pages
- 175-198
- ISSN
- 0001-6322 (ISSN-L)
- 1432-0533
- OpenAlex
- W1984907362
- Cited by
- 225
- References
- 41
- Citations by year
- 2026: 2 · 2025: 3 · 2024: 12 · 2023: 2 · 2022: 2 · 2021: 2 · 2020: 5 · 2019: 1 · 2018: 5 · 2017: 5 · 2016: 1 · 2015: 5 · 2014: 3 · 2013: 5 · 2012: 2
- FWCI
- 4.50
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Acta Neuropathologica (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Neuroscience › Developmental Neuroscience › Neurogenesis and neuroplasticity mechanisms
- Keywords
- Zellweger syndrome, cerebro-hepato-renal syndrome, neuronal migration disorders, cerebral dysgenesis, hypotonia, seizures, developmental delay, white matter degeneration, microglial phagocytosis
- MeSH terms
- Brain Diseases, Cell Migration Inhibition, Cerebral Cortex, Electroencephalography, Female, Growth Disorders, Hearing, Hepatomegaly, Histiocytes, Humans, Infant, Muscle Tonus, Nerve Degeneration, Phagocytosis, Purkinje Cells, Seizures, Sensation, Syndrome, Vision, Ocular
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