Selective neurodegeneration, without neurofibrillary tangles, in a mouse model of Niemann-Pick C disease

Journal of Comparative Neurology

Volumn 433, Issue 3, 2001, Pages 415-425

  German, Dwight C ^a,c   Matthew Quintero, E ^b   Liang, Chang Lin ^a   Ng, Benton ^a   Punia, Surender ^a   Xie, Chonglun ^c   Dietschy, John M ^a  

^a UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

^b BAYLOR COLLEGE OF DENTISTRY   (United States)

^c UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

Author keywords

Cholinergic neurons; Corpus callosum; Glial cells; Prefrontal cortex; Purkinje cells; Stereology; Thalamus

Indexed keywords

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN ATROPHY; CHOLINERGIC NERVE CELL; CORPUS CALLOSUM; DEMYELINATION; ELECTRON MICROSCOPY; FEMALE; GLIA CELL; IMMUNOHISTOCHEMISTRY; MALE; MOUSE; NERVE DEGENERATION; NEUROFIBRILLARY TANGLE; NEUROPATHOLOGY; NIEMANN PICK DISEASE; NONHUMAN; PRIORITY JOURNAL; PURKINJE CELL; STEREOLOGY;

ANIMALS; CHOLINE O-ACETYLTRANSFERASE; DISEASE MODELS, ANIMAL; FEMALE; HISTOCYTOCHEMISTRY; MALE; MICE; MICE, INBRED BALB C; MICROSCOPY, ELECTRON; MUTATION; NERVE DEGENERATION; NEUROFIBRILLARY TANGLES; NEUROGLIA; NEURONS; NIEMANN-PICK DISEASES;

EID: 0035821438     PISSN: 00219967     EISSN: None     Source Type: Journal    
DOI: 10.1002/cne.1149     Document Type: Article

References (59)

1. Paired helical filament tau (PHFtau) in Niemann-Pick type C disease is similar to PHFtau in Alzheimer's disease
2. Type-C Niemann-Pick disease: Low density lipoprotein uptake is associated with premature cholesterol accumulation in the Golgi complex and excessive cholesterol storage in lysosomes
3. Sphingomyelin lipidosis: Niemann-Pick disease
4. Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis
5. The aftermath of boxing
6. Cathepsin D protease mediates programmed cell death induced by interferon-γ Fas/APO-1 and TNF-α
7. Alzheimer-type neuropathology in transgenic mice overexpressing V717F β-amyloid precursor protein
8. 28k in nerve cell nuclei
9. Cathepsin-D is elevated in neurons and microglia in the Niemann-Pick C mouse
10. Niemann-Pick disease type C. Pathological, histochemical, ultrastructural and biochemical studies
11. The coefficient of error of optical fractionator population size estimates: A computer simulation comparing three estimators
12. Tau mutations cause frontotemporal dementias
13. Zur pathogenese des postencephalitischen parkinsonismus
14. Anatomische untersuchungen zur pathogenese des postencephalitischen parkinsonismus
15. Practical considerations for the use of the optical disector in estimating neuronal number
16. Genetic dissection of Alzheimer's disease and related dementias: Amyloid and its relationship to tau
17. Cerebellar degeneration in the Niemann-Pick type C mouse
18. Evidence for a deficit in cholinergic interneurons in the striatum in schizophrenia
19. Tau-based neurofibrillary lesions
20. Paired helical filaments in neurovisceral lipidosis (juvenile dystonic lipidosis)
21. Correlative memory deficits, A-beta elevation, and amyloid plaques in transgenic mice
22. Regulation of a novel pathway for cell death by lysosomal aspartic and cysteine proteinases
23. Presenile appearance of abundant Alzheimer's neurofibrillary tangles without senile plaques in the brain in myotonic dystrophy
24. Neurofibrillary tangles, amyotrophy and progressive motor disturbance in mice expressing mutant (P301L) tau protein
25. 5-enkephalin afferents to midbrain dopaminergic neurons: Light and electron microscopic examination
26. Murine model of Niemann-Pick C disease: Mutation in a cholesterol homeostasis gene
27. Neurofibrillary tangles in Niemann-Pick disease type C
28. Clinical characteristics of a family with chromosome 17-linked disinhibition-dementia-parkinsonism-amyotrophy complex
29. Nucleus basalis (Ch4) and cortical cholinergic innervation in the human brain: Observations based on the distribution of acetylcholinesterase and choline acetyltransferase
30. Lysosomal lipid storage disorder in NCTR-Balb/C mice. 1. Description of the disease and genetics
31. Choline acetyltransferase immunohistochemistry in Alzheimer's disease patients and controls
32. The endosomal-lysosomal system of neurons: New roles
33. Hypoplasia of the corpus callosum in Niemann-Pick type C disease
34. Localization of Niemann-Pick C1 protein in astrocytes: Implication for neuronal degeneration in Niemann-Pick type C disease
35. Subnucleus-specific loss of neurons in medial thalamus of schizophrenics
36. Neurodegeneration in a cholesterol storage disease: A mouse model of Niemann-Pick C disease
37. Neuronal pathology in the nucleus basalis and associated cell groups in senile dementia of the Alzheimer's type: Possible role in cell loss
38. Amyloid degeneration and tuberculosis in the aged
39. Type C Niemann-Pick disease: Lysosomal accumulation and defective intracellular mobilization of low density lipoprotein cholesterol
40. Familial multiple system taupathy with presenile dementia: A disease with abundant neuronal and glial tau filaments
41. Progressive supranuclear palsy
42. Cerebellar involvement in murine sphingomyelinosis: A new model of Niemann-Pick disease
43. Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome
44. Clinical aspects of Niemann-Pick type C disease in the adult
45. Age-associated and cell-type-specific neurofibrillary pathology in transgenic mice expressing the human midsized neurofibrillary subunit
46. Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation
47. Dysmyelination in NCTR-Balb/C mouse mutant with a lysosomal storage disorder
48. New stereological methods for counting neurons
49. Alzheimer's disease and senile dementia: Loss of neurons in the basal forebrain
50. Localization of the gene for rapidly progressive autosomal dominant parkinsonism and dementia with pallido-ponto-nigral degeneration to chromosome 17q21
51. Localization of disinhibition-dementia-parkinsonism-amytrophy complex to 17q21-22
52. Neuronal death and reactive glial changes in the brain of the Niemann-Pick disease type C mouse
53. Reduced number of mediodorsal and anterior thalamic neurons in schizophrenia
54. Cholesterol balance and metabolism in mice with loss of function of Niemann-Pick C protein
55. 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
56. Centripetal cholesterol flow from the extrahepatic organs through the liver is normal in mice with mutated Niemann-Pick type C protein (NPC1)
57. Neurodegeneration in Lurcher mice caused by mutation in delta2 glutamate receptor gene