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

[1]  George Paxinos,et al.  The Mouse Brain in Stereotaxic Coordinates , 2001 .

[2]  D. Burns,et al.  Cholesterol Is Sequestered in the Brains of Mice with Niemann‐Pick Type C Disease but Turnover Is Increased , 2000, Journal of neuropathology and experimental neurology.

[3]  L. Henderson,et al.  Embryonic Striatal Neurons from Niemann-Pick Type C Mice Exhibit Defects in Cholesterol Metabolism and Neurotrophin Responsiveness* , 2000, The Journal of Biological Chemistry.

[4]  R. Proia,et al.  Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse. , 2000, Human molecular genetics.

[5]  Chunjiang Yu,et al.  Role of Niemann-Pick Type C1 Protein in Intracellular Trafficking of Low Density Lipoprotein-derived Cholesterol* , 2000, The Journal of Biological Chemistry.

[6]  Masashi Yamada,et al.  Maternal Pumilio acts together with Nanos in germline development in Drosophila embryos , 1999, Nature Cell Biology.

[7]  J. Dietschy,et al.  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. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[8]  M. Patterson,et al.  The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo* , 1999, The Journal of Biological Chemistry.

[9]  K. Higaki,et al.  Decreased membrane fluidity and unsaturated fatty acids in Niemann-Pick disease type C fibroblasts. , 1998, Biochimica et biophysica acta.

[10]  L. Liscum,et al.  Niemann-Pick disease type C. , 1998, Current opinion in lipidology.

[11]  Kinuko Suzuki,et al.  Recent Advances in Elucidating Niemann‐Pick C Disease , 1998, Brain pathology.

[12]  S. Walkley,et al.  GABAergic neuroaxonal dystrophy and other cytopathological alterations in feline Niemann-Pick disease type C , 1997, Acta Neuropathologica.

[13]  W. Pavan,et al.  Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene. , 1997, Science.

[14]  K. G. Coleman,et al.  Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. , 1997, Science.

[15]  J. Dietschy,et al.  Regional variation in cholesterol synthesis and low density lipoprotein transport in the brain of the fetus, newborn and adult animal , 1997 .

[16]  P. Morell,et al.  Sources of cholesterol during development of the rat fetus and fetal organs. , 1997, Journal of lipid research.

[17]  K. Takeshita,et al.  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 , 1997, Human Genetics.

[18]  C. Rosenfeld,et al.  Brain does not utilize low density lipoprotein-cholesterol during fetal and neonatal development in the sheep. , 1996, Journal of lipid research.

[19]  S. Walkley,et al.  Feline Niemann-Pick disease type C. , 1994, The American journal of pathology.

[20]  W. Strittmatter,et al.  The role of apolipoprotein E in the nervous system. , 1994, Current opinion in lipidology.

[21]  D. Wenger,et al.  Neurological manifestations of Niemann-Pick disease type C in cats. , 1994, Journal of veterinary internal medicine.

[22]  E. Nanba,et al.  A cell line derived from sphingomyelinosis mouse shows alterations in intracellular cholesterol metabolism similar to those in type C Niemann-Pick disease. , 1992, Cell structure and function.

[23]  K. Mikoshiba,et al.  Immunohistochemical expression of P400 protein in purkinje cells of sphingomyelinosis mouse , 1991, Brain and Development.

[24]  D. Cogan,et al.  Clinical spectrum of Niemann‐Pick disease type C , 1989, Neurology.

[25]  Haruomi Nakamura,et al.  Cerebellar Involvement in Murine Sphingomyelinosis: A New Model of Niemann-Pick Disease , 1988, Journal of neuropathology and experimental neurology.

[26]  R. Mahley,et al.  Astrocytes synthesize apolipoprotein E and metabolize apolipoprotein E-containing lipoproteins. , 1987, Biochimica et biophysica acta.

[27]  S. Ikehara,et al.  A mouse model for Niemann-Pick disease. Influence of genetic background on disease expression in spm/spm mice. , 1986, The Journal of heredity.

[28]  M. Alexander,et al.  Principles of Neural Science , 1981 .

[29]  M. Vanier Biochemical studies in Niemann-Pick disease. I. Major sphingolipids of liver and spleen. , 1983, Biochimica et biophysica acta.

[30]  T. Kitagawa,et al.  Sphingomyelinosis, a new mutation in the mouse: a model of Niemann-Pick disease in humans. , 1982, The Journal of heredity.

[31]  H. Shio,et al.  Lysosome lipid storage disorder in NCTR-BALB/c mice. I. Description of the disease and genetics. , 1982, The American journal of pathology.

[32]  J. Martín,et al.  Niemann-Pick disease. Morphologic and biochemical studies in the visceral form with late central nervous system involvement (Crocker's group C). , 1969, Archives of neurology.

[33]  G. Millat,et al.  Niemann-Pick disease type C. , 2003, Clinical Genetics.

[34]  R. Palmiter,et al.  Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport , 1999, Nature Cell Biology.