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15. PRNP Mutation (G131V) Associated With Gerstmann-Sträussler-Scheinker Disease.

32. Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutations.

33. Additional copies of the proteolipid protein gene causing Pelizaeus-Merzbacher disease arise by separate integration into the X chromosome.

34. A cell cycle alteration precedes apoptosis of granule cell precursors in the weaver mouse cerebellum.

35. Different mutations in the same codon of the proteolipid protein gene, PLP, may help in correlating genotype with phenotype in Pelizaeus-Merzbacher disease/X-linked spastic paraplegia (PMD/SPG2).

36. Hypothalamic-pituitary-gonadal axis in the mutant weaver mouse.

37. Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity.

38. Characterization of murine Girk2 transcript isoforms: structure and differential expression.

39. An antibody raised against a conserved sequence of the prion protein recognizes pathological isoforms in human and animal prion diseases, including Creutzfeldt-Jakob disease and bovine spongiform encephalopathy.

40. X-linked spastic paraplegia due to a mutation (C506T; Ser169Phe) in exon 4 of the proteolipid protein gene (PLP).

41. Diverse cell death pathways result from a single missense mutation in weaver mouse.

42. A male child with the rumpshaker mutation, X-linked spastic paraplegia/Pelizaeus-Merzbacher disease and lysinuria.

43. Multiplex PCR: critical parameters and step-by-step protocol.

44. Family with Pelizaeus-Merzbacher disease/X-linked spastic paraplegia and a nonsense mutation in exon 6 of the proteolipid protein gene.

45. In situ hybridization analysis of Girk2 expression in the developing central nervous system in normal and weaver mice.

46. Proteolipid protein is necessary in peripheral as well as central myelin.

47. Nonsense mutation in exon 3 of the proteolipid protein gene (PLP) in a family with an unusual form of Pelizaeus-Merzbacher disease.

48. Neuropsychological function in patients with Gerstmann-Sträussler-Scheinker disease from the Indiana kindred (F198S).

49. Gerstmann-Sträussler-Scheinker disease with the PRNP P102L mutation and valine at codon 129.

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