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Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the "twitcher" mouse model of globoid cell leukodystrophy.
Tominaga K, Matsuda J, Kido M, Naito E, Yokota I, Toida K, Ishimura K, Suzuki K, Kuroda Y. Tominaga K, et al. J Neurosci Res. 2004 Aug 15;77(4):507-16. doi: 10.1002/jnr.20190. J Neurosci Res. 2004. PMID: 15264220
The twitcher mouse is well known as a naturally occurring authentic mouse model of human globoid cell leukodystrophy (GLD; Krabbe disease) due to genetic deficiency of lysosomal galactosylceramidase. ...In the hippocampus, the neuronal cell death occur …
The twitcher mouse is well known as a naturally occurring authentic mouse model of human globoid cell leukodystrophy (G …
CSF1R mutations link POLD and HDLS as a single disease entity.
Nicholson AM, Baker MC, Finch NA, Rutherford NJ, Wider C, Graff-Radford NR, Nelson PT, Clark HB, Wszolek ZK, Dickson DW, Knopman DS, Rademakers R. Nicholson AM, et al. Neurology. 2013 Mar 12;80(11):1033-40. doi: 10.1212/WNL.0b013e31828726a7. Epub 2013 Feb 13. Neurology. 2013. PMID: 23408870 Free PMC article.
OBJECTIVE: Pigmented orthochromatic leukodystrophy (POLD) and hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) are rare neurodegenerative disorders characterized by cerebral white matter abnormalities, myelin loss, and axonal swellings. ...RESULTS: We id …
OBJECTIVE: Pigmented orthochromatic leukodystrophy (POLD) and hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) are …