Neurofibromatosis type 1: II. Answers from animal models

Journal of Neuroscience Research

Volumn 65, Issue 3, 2001, Pages 191-194

  Lakkis, Maha M ^a,b   Tennekoon, Gihan I ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b 502 Abramson Building ^*  (United States)

Author keywords

[No Author keywords available]

Indexed keywords

NEUROFIBROMIN;

ALLELE; ANIMAL EXPERIMENT; ANIMAL MODEL; GENE DISRUPTION; GENE EXPRESSION REGULATION; GENE INACTIVATION; GENE MUTATION; GENETIC PREDISPOSITION; HETEROZYGOSITY LOSS; MOUSE; NERVE SHEATH TUMOR; NEUROFIBROMA; NEUROFIBROMATOSIS; NONHUMAN; PRIORITY JOURNAL; SHORT SURVEY;

ANIMALS; BRAIN; CELL TRANSFORMATION, NEOPLASTIC; CHIMERA; DISEASE MODELS, ANIMAL; DROSOPHILA MELANOGASTER; FIBROBLASTS; GANGLIA, SYMPATHETIC; GENE TARGETING; GENES, LETHAL; GENES, NEUROFIBROMATOSIS 1; GENES, P53; GENOTYPE; GLIOSIS; HEART DEFECTS, CONGENITAL; HUMANS; INSECT PROTEINS; LEARNING DISORDERS; LOSS OF HETEROZYGOSITY; MICE; MICE, KNOCKOUT; MICE, MUTANT STRAINS; NEOPLASTIC SYNDROMES, HEREDITARY; NERVE TISSUE PROTEINS; NEURAL CREST; NEUROFIBROMATOSIS 1; NEUROFIBROMIN 1; PHENOTYPE; RATS; SCHWANN CELLS;

EID: 0035424635     PISSN: 03604012     EISSN: None     Source Type: Journal    
DOI: 10.1002/jnr.1142     Document Type: Short Survey

References (38)

1. The Nf1 tumor suppressor regulates mouse skin wound healing, fibroblast proliferation, and collagen deposited by fibroblasts
2. Loss of NF1 results in activation of the Ras signaling pathway and leads to aberrant growth in haematopoietic cells
3. Targeted disruption of the neurofibromatosis type-1 gene leads to developmental abnormalities in heart and various neural crest-derived tissues
4. Neurobehavioral profiles of children with neurofibromatosis type 1 referred for learning disabilities are sex-specific
5. Mouse models of tumor development in neurofibromatosis type 1
6. The protein product of the neurofibromatosis type 1 gene is expressed at highest abundance in neurons, Schwann cells, and oligodendrocytes
7. Physical mapping of the von Recklinghausen neurofibromatosis region on chromosome 17
8. Requirement of Drosophila NF1 for activation of adenylyl cyclase by PACAP38-like neuropeptides
9. Modulation of the neurofibromatosis type 1 gene product, neurofibromin, during Schwann cell differentiation
10. Tumor predisposition in mice heterozygous for a targeted mutation in Nf1
11. Schwann cells from neurofibromin deficient mice exhibit activation of p21ras, inhibition of cell proliferation, and morphological changes
12. Nf1-deficient mouse Schwann cells are angiogenic and invasive and can be induced to hyperproliferate: Reversion of some phenotypes by an inhibitor of farnesyl protein transferase
13. Neurofibromin modulation of ras activity is required for normal endocardial-mesenchymal transformation in the developing heart
14. Neurofibromatosis type 1: I
15. Neurofibromin deficiency in mice causes exencephaly and is a modifier for Splotch neural tube defects
16. The neurofibromatosis type 1 (NF1) tumor suppressor gene and myeloid leukemia
17. Cardiac abnormalities in neurofibromatosis
18. cDNA cloning of the type 1 neurofibromatosis gene: Complete sequence of the NF1 gene
19. Neurofibromin expression and astrogliosis in neurofibromatosis (type 1) brains
20. Cognitive function and academic performance in children with neurofibromatosis type 1
21. Cognitive impairment in neurofibromatosis type 1
22. Collagens in neurofibromas and neurofibroma cell cultures
23. Cellular differentiation and expression of matrix genes in type 1 neurofibromatosis
24. Von Recklinghausen neurofibromatosis
25. Region-specific astrogliosis in brains of mice heterozygous for mutations in the neurofibromatosis type 1 (Nf1) tumor suppressor
26. Neurofibromin-deficient fibroblasts fail to form perineurium in vitro
27. 0 expression and myelination
28. MRI morphometric analysis and neuropsychological function in patients with neurofibromatosis
29. Linkage analysis in von Recklinghausen neurofibromatosis (NF1) with DNA markers for chromosome 17
30. A mouse model for the learning and memory deficits associated with neurofibromatosis type 1
31. Tandem duplication within a neurofibromatosis type 1 (NF1) gene exon in a family with features of Watson syndrome and Noonan syndrome
32. Rescue of a Drosophila NF1 mutant phenotype by protein kinase A
33. Sympathetic neuron survival and proliferation are prolonged by loss of p53 and neurofibromin
34. Loss of neurofibromin results in neurotrophin-independent survival of embryonic sensory and sympathetic neurons
35. Mouse tumor model for neurofibromatosis type 1
36. Type 1 neurofibromatosis gene: Identification of a large transcript disrupted in three NF1 patients
37. Up-regulation of tumor suppressor protein neurofibromin in normal human wound healing and in vitro evidence for platelet derived growth factor (PDGF) and transforming growth factor-beta 1 (TGF-β) elicited increase in neurofibromin mRNA steady-state levels in dermal fibroblasts
38. Nf1 regulates hematopoietic progenitor cell growth and ras signaling in response to multiple cytokines