Progressive neuronal loss in the ventral posterior lateral and medial nuclei of thalamus in Niemann-Pick disease type C mouse brain

Brain and Development

Volumn 23, Issue 5, 2001, Pages 288-297

  Yamada, Atsuko ^a   Saji, Makoto ^a   Ukita, Yoko ^a   Shinoda, Yoko ^a   Taniguchi, Miyako ^a   Higaki, Katsumi ^a   Ninomiya, Haruaki ^a   Ohno, Kousaku ^a  

^a TOTTORI UNIVERSITY   (Japan)

Author keywords

Cholesterol; Filipin; Neuronal cell loss; Niemann Pick disease type C; Purkinje cell; Thalamus

Indexed keywords

CHOLESTEROL; FILIPIN; GLYCOPHOSPHOLIPID;

ANIMAL CELL; ARTICLE; BASAL GANGLION; CELL LOSS; CELL MUTANT; CELL STRAIN; CELL SWELLING; CONTROLLED STUDY; FEMALE; HIPPOCAMPUS; LIPID BRAIN LEVEL; MALE; MOUSE; NEOCORTEX; NERVE CELL; NIEMANN PICK DISEASE; NONHUMAN; PURKINJE CELL; PYRIFORM CORTEX; STAINING; THALAMUS NUCLEUS; TISSUE LEVEL;

AGING; ANIMALS; CELL SIZE; CHOLESTEROL; DETERGENTS; FILIPIN; IMMUNOHISTOCHEMISTRY; LIVER; MACROPHAGES; MICE; MICE, NEUROLOGIC MUTANTS; NERVE DEGENERATION; NEURONS; NIEMANN-PICK DISEASES; POLYETHYLENE GLYCOLS; PURKINJE CELLS; VENTRAL THALAMIC NUCLEI;

EID: 0034897464     PISSN: 03877604     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0387-7604(01)00209-1     Document Type: Article

References (41)

1. Niemann-Pick disease type C
2. Recent advances in elucidating of Niemann-Pick C disease
3. Sphingomyelinosis, a new mutation in the mouse: A model of Niemann Pick disease in humans
4. A mouse model for Niemann-Pick disease. Influence of genetic background on disease expression in spm/spm mice
5. Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis
6. Murine model of Niemann-Pick C disease: Mutation in a cholesterol homeostasis gene
7. A C57BL/KsJ mouse model of Niemann-Pick disease (spm) belongs to the same complementation group as the major childhood type of Niemann-Pick disease type C
8. Niemann-Pick disease type C
9. The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo
10. Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport
11. Role of Niemann-Pick type C1 protein in intracellular trafficking of low density lipoprotein derived-cholesterol
12. Cerebellar involvement in murine sphingomyelinosis: A new model of Niemann-Pick disease
13. A cell line derived from sphingomyelinosis mouse shows alterations in intracellular cholesterol metabolism similar to those in type C Niemann-Pick disease
14. Decreased membrane fluidity and unsaturated fatty acids in Niemann-Pick disease type C fibroblasts
15. Immunohistochemical expression of P400 protein in Purkinje cells of sphingomyelinosis mouse
16. Cerebellar degeneration in the Niemann-Pick type C mouse
17. Niemann-Pick disease type C. Study on the nature of the cerebral storage process
18. Neurofibrillary tangles in Niemann-Pick disease type C
19. Feline Niemann-Pick disease type C
20. Neurological manifestations of Niemann-Pick disease type C in cats
21. GABAergic neuroaxonal dystrophy and other cytopathological alterations in feline Niemann-Pick disease type C
22. Anatomy of the somatic sensory system
23. Trigeminal system
24. Clinical spectrum of Niemann-Pick disease type C
25. Niemann-Pick disease. Morphologic and biochemical studies in the visceral form with late central nervous system involvement (Crocker's group C)
26. A case of juvenile lipidosis: Electron microscopic, histochemical and biochemical studies
27. Biochemical studies in Niemann-Pick disease. I. Major sphingolipids of liver and spleen
28. Lysosome lipid storage disorder in NCTR-BALB/c mice. I. Description of the disease and genetics
29. Cholesterol and oxygenated cholesterol concentrations are markedly elevated in peripheral tissue but not in brain from mice with the Niemann-Pick C phenotype
30. Niemann-Pick disease type C. Study on the nature of the cerebral storage process
31. Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse
32. Cholesterol is sequestered in the brains of mice with Niemann-Pick type C disease but turnover is increased
33. Brain does not utilize low density lipoprotein -cholesterol during fetal and neonatal development in the sheep
34. Regional variation in cholesterol synthesis and low-density lipoprotein cholesterol in the brain of the fetus, newborn and adults animals
35. Sources of cholesterol during development of the rat fetus and fetal organs
36. Astrocytes synthesize apolipoprotein E and metabolize apolipoprotein E-containing lipoproteins
37. The role of apolipoprotein E in the nervous system
38. Cholesterol accumulation in tissues of the Niemann-Pick type C mouse is determined by the rate of lipoprotein-cholesterol uptake through the coated-pit pathway in each organ
39. Embryonic striatal neurons from Niemann-Pick type C mice exhibit defects in cholesterol metabolism and neurotrophin responsiveness