Cerebellar ganglioside abnormalities in <i>pcd</i> mutant mice

Seyfried, Thomas N.; Yu, Robert K.

doi:10.1002/jnr.490260113

Cited by 16 publications

(10 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Although gangliosides GT1a and LD1 comigrate on the HPTLC in this solvent system, previous studies showed that LD1 [disialosylparagloboside (disialosyl‐nLc4Cer)] is the major ganglioside in this band (Chou et al. 1990; Seyfried and Yu 1990). In addition, asialo‐GM2 (GA2) and asialo‐GM1 (GA1) were elevated in the Hexb −/− and the β‐gal −/− mice, respectively (Fig.…”

Section: Methodsmentioning

confidence: 75%

See 1 more Smart Citation

Neurochemical, morphological, and neurophysiological abnormalities in retinas of Sandhoff and GM1 gangliosidosis mice

Denny

Alroy

Pawlyk

et al. 2007

Journal of Neurochemistry

View full text Add to dashboard Cite

Retinal abnormalities are well documented in patients with ganglioside storage diseases. The total content and distribution of retinal glycosphingolipids was studied for the first time in control mice and in Sandhoff disease (SD) and GM1 gangliosidosis mice. Light and electron microscopy of the SD and the GM1 retinas revealed storage in ganglion cells. Similar to previous findings in rat retina, GD3 was the major ganglioside in mouse retina, while GM2 and GM1 were minor species. Total ganglioside content was 44% and 40% higher in the SD and the GM1 retinas, respectively, than in the control retinas. Furthermore, GM2 and GM1 content were 11-fold and 51-fold higher in the SD and the GM1 retinas than in the control retinas, respectively. High concentrations of asialo-GM2 and asialo-GM1 were found in the SD and the GM1 retinas, respectively, but were undetectable in the control retinas. The GSL abnormalities in the SD and the GM1 retinas reflect significant reductions in b-hexosaminidase and b-galactosidase enzyme activities, respectively. Although electroretinograms appeared normal in the SD and the GM1 mice, visual evoked potentials were subnormal in both mutants, indicating visual impairments. Our findings present a model system for assessing retinal pathobiology and therapies for the gangliosidoses.

show abstract

Section: Methodsmentioning

confidence: 75%

“…1987; Chou et al. 1990Seyfried and Yu 1990). Interestingly, these mice also express retinal degeneration and visual impairment (Mullen et al.…”

Section: Discussionmentioning

confidence: 99%

Neurochemical, morphological, and neurophysiological abnormalities in retinas of Sandhoff and GM1 gangliosidosis mice

Denny

Alroy

Pawlyk

et al. 2007

Journal of Neurochemistry

View full text Add to dashboard Cite

show abstract

“…Gangliosides GD1a and GT1b are enriched in neuronal and synaptic membranes, whereas ganglioside GD3 is enriched in reactive glial cells and is a good marker for neurodegeneration (18-21). In the mouse cerebellum, GD1a is enriched in granule cells while LD1/GT1a is enriched in Purkinje cells (22, 23). Changes in these gangliosides are indicative of changes in granule cells and Purkinje cells, respectively (19, 20, 22, 24).…”

Section: Introductionmentioning

confidence: 99%

Brain Lipid Analysis in Mice with Rett Syndrome

et al. 2008

View full text Add to dashboard Cite

Rett syndrome (RS) is an X-linked neurodevelopmental disorder mostly involving mutations in the gene for methyl-CpG-binding protein 2 (MECP2). Ganglioside abnormalities were previously found in cerebrum and cerebellum in RS patients. We evaluated total lipid distribution in cerebrum/brainstem, hippocampus, and cerebellum in male mice carrying either the Mecp2tm1.1Bird knockout mutation or the Mecp2308/y deletion mutation. The concentration of the neuronal enriched ganglioside GD1a was significantly lower in the cerebrum/brainstem of Mecp2tm1.1Bird mice than in that of age matched controls, but was not reduced in the Mecp2308/y mice. No other differences in brain lipid content, including myelin-enriched cerebrosides, were detected in mice with either type of Mecp2 mutation. These findings indicate that the poor motor performance previously reported in the RS mutant mice is not associated with major brain lipid abnormalities and that most previous brain lipid abnormalities observed in RS patients were not observed in the Mecp2tm1.1Bird or the Mecp2308/y RS mice.

show abstract

“…It has been suggested that an increased shedding of membrane fragments from myelin leads to an increased cerebrospinal fluid (CSF) sul fatide concentration. In vascular dementia, an increased concentration of sulfatide in CSF has been found, proba bly reflecting demyelination [19,20], Ganglioside GD3 is a minor ganglioside in the normal brain, but increases in reactive astrocytes after different forms of trauma [21] and neuronal degeneration [22], Therefore, ganglioside GD3 has a potential as a biochemi cal marker for reactive gliosis.…”

Section: Introductionmentioning

confidence: 99%

Gangliosides and Sulfatide in Cerebrospinal Fluid in Leukoaraiosis

Tarvonen-Schröder

Blennow

Lekman

et al. 1997

Dement Geriatr Cogn Disord

View full text Add to dashboard Cite

The aim of the study was to evaluate gangliosides and sulfatide in cerebrospinal fluid (CSF) as markers for neuronal degeneration, gliosis, and demyelination in leukoaraiosis (LA). Lumbar CSF samples were taken from 37 elderly subjects with LA on computed tomography (CT). Patients with other pathology than LA or infarction on CT were excluded. In addition, CSF samples were collected from 16 elderly reference subjects without any neurological disease. Gangliosides GM1, GD1a, GD1b, GT1b, GD3, and sulfatide were determined. The concentration of the individual gangliosides and sulfatide showed no correlation with age. Gangliosides GD1b, GT1b, and GD3 were elevated in patients with mild LA compared to controls and patients with moderate or severe LA. GD1a was elevated in patients with mild LA compared to those with moderate LA. The concentration of sulfatide did not differ between the groups. When the patients were grouped in accordance to whether they had had cerebral infarction or not, differences between the groups were not found in the concentrations of any gangliosides and sulfatide. In conclusion, the analysis of CSF markers suggests that neuronal degeneration and gliosis predominate in the early stage of LA.

show abstract

Cerebellar ganglioside abnormalities in pcd mutant mice

Cited by 16 publications

References 29 publications

Neurochemical, morphological, and neurophysiological abnormalities in retinas of Sandhoff and GM1 gangliosidosis mice

Neurochemical, morphological, and neurophysiological abnormalities in retinas of Sandhoff and GM1 gangliosidosis mice

Brain Lipid Analysis in Mice with Rett Syndrome

Gangliosides and Sulfatide in Cerebrospinal Fluid in Leukoaraiosis

Contact Info

Product

Resources

About