Establishment and characterization of immortalized Schwann cells from murine model of Niemann-Pick disease type C (spm/spm)

Journal of the Peripheral Nervous System

Volumn 6, Issue 2, 2001, Pages 85-94

  Watabe, Kazuhiko ^a   Ida, Hiroyuki ^b   Uehara, Keiko ^b   Oyanagi, Kiyomitsu ^a   Sakamoto, Tsuyoshi ^a,b   Tanaka, Junichi ^b   Garver, William S ^c   Miyawaki, Shigeki ^d   Ohno, Kousaku ^e   Eto, Yoshikatsu ^b  

^a TOKYO METROPOLITAN INSTITUTE OF MEDICAL SCIENCE   (Japan)

^b JIKEI UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^c UNIVERSITY OF ARIZONA COLLEGE OF MEDICINE   (United States)

^d NIPPON SHINYAKU CO LTD   (Japan)

^e TOTTORI UNIVERSITY   (Japan)

Author keywords

Dorsal root ganglia; Niemann Pick disease type C; NPC1; Schwann cell; Tissue culture

Indexed keywords

ACRIFLAVINE; CERAMIDE; CHOLESTEROL; GANGLIOSIDE GM2; MIGLUSTAT;

ANIMAL CELL; ARTICLE; ELECTRON MICROSCOPY; ENDOSOME; IMMUNOFLUORESCENCE; IMMUNOREACTIVITY; LYSOSOME; MOUSE; NERVE DEGENERATION; NIEMANN PICK DISEASE; NONHUMAN; NUCLEOTIDE SEQUENCE; PATHOGENESIS; PHENOTYPE; PRIORITY JOURNAL; SCHWANN CELL; SPINAL GANGLION;

ANIMALS; BLOTTING, WESTERN; CELLS, CULTURED; CYTOLOGICAL TECHNIQUES; FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT; GANGLIA, SPINAL; MICE; NIEMANN-PICK DISEASES; PERIPHERAL NERVES; PHENOTYPE; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; RNA, MESSENGER; SCHWANN CELLS;

EID: 0034963585     PISSN: 10859489     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1529-8027.2001.01012.x     Document Type: Article

References (48)

1. Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis
2. Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis
3. Fate of endogenously synthesized cholesterol in Niemann-Pick type C cells
4. Transmembrane molecular pump activity of Niemann-Pick C1 protein
5. Pharmacological and genetic modifications of somatic cholesterol do not alter the course of CNS disease in Niemann-Pick C mice
6. Expression of Niemann-Pick type C transcript in rodent cerebellum in vivo and in vitro
7. Localization of the murine Niemann-Pick C1 protein to two distinct intracellular compartments
8. Nerve biopsy findings in Niemann-Pick type II (NPC)
9. Embryonic striatal neurons from Niemann-Pick type C mice exhibit defects in cholesterol metabolism and neurotrophin responsiveness
10. Pathology of Niemann-Pick type C: Studies of murine mutants
11. Peripheral nerve pathology in Niemann-Pick type C mouse
12. Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin
13. Cloning of complementary DNA for GAP-43, a neuronal growth-related protein
14. Generation of one set of monoclonal antibodies specific for a-pathway ganglio-series gangliosides
15. Niemann-Pick disease type C
16. Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse
17. Murine model of Niemann-Pick C disease: Mutation in a cholesterol homeostasis gene
18. A growth arrest specific (gas) gene codes for a membrane protein
19. Lack of repricative senescence in normal rodent glia
20. The neurobiology of Schwann cells
21. Sphingomyelinosis, a new mutation in the mouse. A model of Niemann-Pick disease in humans
22. Neural adhesion molecule L1 as a member of the immunoglobulin superfamily with binding domains similar to fibronectin
23. Identification of HE1 as the second gene of Niemann-Pick C disease
24. A cell line derived from sphingomyelinosis mouse shows alterations in intracellular cholesterol metabolism similar to those in type C Niemann-Pick disease
25. Impaired cholesterol esterification in primary brain cultures of the lysosomal cholesterol storage disorder (LCSD) mouse mutant
26. 25-Hydroxycholesterol induces reorganization of lysosomes in normal but not Niemann-Pick disease type C astrocytes
27. A lysosomal storage disorder in mice characterized by a dual deficiency of sphingomyelinase and glucocerebrosidase
28. A genetic storage disorder in BALB/c mice with a metabolic block in esterification of exogenous cholesterol
29. Niemann-Pick disease type C: A cellular cholesterol lipidosis
30. Quantitative competitive polymerase chain reaction for accurate quantitation of HIV DNA and RNA species
31. N-Butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis
32. Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin
33. Gene transfer and molecular cloning of the rat nerve growth factor receptor
34. A lysosomal storage disorder in mice: A model of Niemann-Pick disease
35. Jamming the endosomal system: Lipid rafts and lysosomal storage diseases
36. Increased angiotensin converting enzyme expression in the failing human heart
37. Cellular cholesterol storage in Niemann-Pick disease type C mouse is associated with increased expression and defective processing of apolipoprotein D
38. Cerebellar involvement in murine sphingomyelinosis: A new model of Niemann-Pick disease
39. Recent advances in elucidating Niemann-Pick C disease
40. Transfection and stable transformation of adult mouse Schwann cells with SV-40 large T antigen gene
41. Cerebral and spinal involvements in sphingomyelinosis mouse
42. Mitogenic effects of platelet-derived growth factor, fibroblast growth factor, transforming growth factor-β, and heparin-binding serum factor for adult mouse Schwann cells
43. Spontaneously immortalized adult mouse Schwann cells secrete autocrine and paracrine growth-promoting activities
44. Establishment of spontaneously immortalized Schwann cells from murine models of Niemann-Pick disease type C (NPC; spm) and globoid cell leukodystrophy (GCL; twitcher)
45. A possible same genetic defect in two Niemann-Pick disease model mice
46. Accumulation of GM2 ganglioside in Niemann-Pick disease type C fibroblasts
47. DNA sequence, genomic organization, and chromosomal localization of the mouse peripheral myelin protein zero gene: Identification of polymorphic alleles
48. Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations