Early onset of axonal degeneration in double (plp-/-mag-/-) and hypomyelinosis in triple (plp-/-mbp-/-mag-/-) mutant mice

Journal of Neuroscience

Volumn 20, Issue 14, 2000, Pages 5225-5233

  Uschkureit, Thomas ^a   Spörkel, Olaf ^a   Stracke, Jens ^a   Büssow, Heinrich ^b   Stoffel, Wilhelm ^a  

^a UNIVERSITY OF COLOGNE   (Germany)

^b UNIVERSITY OF BONN   (Germany)

Author keywords

Behavioral tests; Double mutants; Myelin lipids; Myelin proteins; Triple mutant; Ultrastructure of myelin

Indexed keywords

MYELIN ASSOCIATED GLYCOPROTEIN; MYELIN BASIC PROTEIN; PROTEOLIPID PROTEIN;

ANIMAL BEHAVIOR; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; COGNITION; DISEASE ASSOCIATION; HISTOPATHOLOGY; LIFESPAN; MOTOR COORDINATION; MOUSE; MYELINATION; NERVE FIBER DEGENERATION; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEFICIENCY;

EID: 0343953472     PISSN: 02706474     EISSN: None     Source Type: Journal    
DOI: 10.1523/jneurosci.20-14-05225.2000     Document Type: Article

References (54)

1. Archer DR, Cuddon PA, Lipsitz D, Duncan LD (1997) Myelination of the canine central nervous system by glial cell transplantation: a model for repair of human myelin disease. Nat Med 3:54-59.
2. Arquint M, Roder J, Chia LS, Down J, Wilkinson D, Bayley H, Braun P, Dunn R (1987) Molecular cloning and primary structure of myelin-associated glycoprotein. Proc Natl Acad Sci USA 84:600-604.
3. Bird TD, Farrell DF, Sumi SM (1978) Brain lipid composition of the shiverer mouse: (genetic defect in myelin development). J Neurochem 31:387-391.
4. Bligh E, Dyer W (1959) A rapid method of total lipid extraction and purification. Can J Biochem Physiol 37:911-917.
5. Boison D, Stoffel W (1989) Myelin-deficient rat: a point mutation in exon III (A - C, Thr75 - Pro) of the myelin proteolipid protein causes dysmyelination and oligodendrocyte death. EMBO J 8:3295-302.
6. Boison D, Stoffel W (1994) Disruption of the compacted myelin sheath of axons of the central nervous system in proteolipid protein-deficient mice. Proc Natl Acad Sci USA 91:11709-11713.
7. Boison D, Büssow H, D'Urso D, Muller HW, Stoffel W (1995) Adhesive properties of proteolipid protein are responsible for the compaction of CNS myelin sheaths. J Neurosci 15:5502-5513.
8. Bongarzone ER, Campagnoni CW, Kampf K, Jacobs EC, Handley VW, Schonmann V, Campagnoni AT (1999) Identification of a new exon in the myelin proteolipid protein gene encoding novel protein isoforms that are restricted to the somata of oligodendrocytes and neurons [In Process Citation]. J Neurosci 19:8349-8357.
9. Brustle O, Jones KN, Learish RD, Karram K, Choudhary K, Wiestler OD, Duncan ID, McKay RD (1999) Embryonic stem cell-derived glial precursors: a source of myelinating transplants [see comments]. Science 285:754-756.
10. Büssow H (1978) Schwann cell myelin ensheathing CNS axons in the nerve fibre layer of the cat retina. J Neurocytol 7:207-214.
11. Cammer W, Kahn S, Zimmerman T (1984) Biochemical abnormalities in spinal cord myelin and CNS homogenates in heterozygotes affected by the shiverer mutation. J Neurochem 42:1372-1378.
12. Carenini S, Montag D, Cremer H, Schachner M, Martini R (1997) Absence of the myelin-associated glycoprotein (MAG) and the neural cell adhesion molecule (N-CAM) interferes with the maintenance, but not with the formation of peripheral myelin. Cell Tissue Res 287:3-9.
13. Chernoff GF (1981) Shiverer: an autosomal recessive mutant mouse with myelin deficiency. J Hered 72:128.
14. Chomczynski P, Sacchi N (1987) Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction. Anal Biochem 162:156-159.
15. Fruttiger M, Montag D, Schachner M, Martini R (1995) Crucial role for the myelin-associated glycoprotein in the maintenance of axon-myelin integrity. Eur J Neurosci 7:511-515.
16. Fujita N, Kemper A, Dupree J, Nakayasu H, Bartsch U, Schachner M, Maeda N, Suzuki K, Popko B (1998) The cytoplasmic domain of the large myelin-associated glycoprotein isoform is needed for proper CNS but not peripheral nervous system myelination. J Neurosci 18:1970-1978.
17. Gould RM, Byrd AL, Barbarese E (1995) The number of Schmidt-Lanterman incisures is more than doubled in shiverer PNS myelin sheaths. J Neurocytol 24:85-98.
18. Griffiths I, Klugmann M, Andersen T, Yool D, Thomson C, Schwab MH, Schneider A, Zimmermann F, McCulloch M, Nadon N, Nave KA (1998) Axonal swellings and degeneration in mice lacking the major proteolipid of myelin. Science 280:1610-1613.
19. Inuzuka T, Fujita N, Sato S, Baba H, Nakano R, Ishiguro H, Miyatake T (1991) Expression of the large myelin-associated glycoprotein isoform during the development in the mouse peripheral nervous system. Brain Res 562:173-175.
20. Iwamori M, Harpin ML, Lachapelle F, Baumann N (1985) Brain gangliosides of quaking and shiverer mutants: qualitative and quantitative changes of monosialogangliosides in the quaking brain. J Neurochem 45:73-78.
21. Johnson RS, Roder JC, Riordan JR (1995) Over-expression of the DM-20 myelin proteolipid causes central nervous system demyelination in transgenic mice. J Neurochem 64:967-976.
22. Kagawa T, Ikenaka K, Inoue Y, Kuriyama S, Tsujii T, Nakao J, Nakajima K, Aruga J, Okano H, Mikoshiba K (1994) Glial cell degeneration and hypomyelination caused by overexpression of myelin proteolipid protein gene. Neuron 13:427-442.
23. Klapdor K, van der Staay FJ (1996) The Morris water-escape task in mice: strain differences and effects of intra-maze contrast and brightness. Physiol Behav 60:1247-1254.
24. Klapdor K, van der Staay FJ (1998) Repeated acquisition of a spatial navigation task in mice: effects of spacing of trials and of unilateral middle cerebral artery occlusion. Physiol Behav 63:903-909.
25. Klugmann M, Schwab MH, Puhlhofer A, Schneider A, Zimmermann F, Griffiths IR, Nave KA (1997) Assembly of CNS myelin in the absence of proteolipid protein. Neuron 18:59-70.
26. Kuhn PL, Petroulakis E, Zazanis GA, McKinnon RD (1995) Motor function analysis of myelin mutant mice using a rotarod. Int J Dev Neurosci 13:715-722.
27. Lai C, Brow MA, Nave KA, Noronha AB, Quarles RH, Bloom FE, Milner RJ, Sutcliffe JG (1987) Two forms of 1B236/myelin-associated glycoprotein, a cell adhesion molecule for postnatal neural development, are produced by alternative splicing. Proc Natl Acad Sci USA 84:4337-4341.
28. Lees M, Brostoff SW (1984) Proteins of myelin. New York: Plenum.
29. Li C, Tropak MB, Gerlai R, Clapoff S, Abramow-Newerly W, Trapp B, Peterson A, Roder J (1994) Myelination in the absence of myelin-associated glycoprotein. Nature 369:747-750.
30. Molineaux SM, Engh H, de Ferra F, Hudson L, Lazzarini RA (1986) Recombination within the myelin basic protein gene created the dysmyelinating shiverer mouse mutation. Proc Natl Acad Sci USA 83:7542-7546.
31. Montag D, Giese KP, Bartsch U, Martini R, Lang Y, Bluthmann H, Karthigasan J, Kirschner DA, Wintergerst ES, Nave KA (1994) Mice deficient for the myelin-associated glycoprotein show subtle abnormalities in myelin. Neuron 13:229-246.
32. Morris R (1984) Developments of a water-maze procedure for studying spatial learning in the rat. J Neurosci Methods 11:47-60.
33. Nagara H, Suzuki K, Tateishi J (1983) Radial component of central myelin in shiverer mouse. Brain Res 263:336-339.
34. Nave KA, Lai C, Bloom FE, Milner RJ (1986) Jimpy mutant mouse: a 74-base deletion in the mRNA for myelin proteolipid protein and evidence for a primary defect in RNA splicing. Proc Natl Acad Sci USA 83:9264-9268.
35. Norton WT, Poduslo SE (1973) Myelination in rat brain: method of myelin isolation. J Neurochem 21:749-757.
36. Omlin FX, Webster HD, Palkovits CG, Cohen SR (1982) Immunocytochemical localization of basic protein in major dense line regions of central and peripheral myelin. J Cell Biol 95:242-248.
37. Pedraza L, Frey AB, Hempstead BL, Colman DR, Salzer JL (1991) Differential expression of MAG isoforms during development. J Neurosci Res 29:141-148.
38. Raff MC, Mirsky R, Fields KL, Lisak RP, Dorfman SH, Silberberg DH, Gregson NA, Leibowitz S, Kennedy MC (1978) Galactocerebroside is a specific cell-surface antigenic marker for oligodendrocytes in culture. Nature 274:813-816.
39. Readhead C, Schneider A, Griffiths I, Nave KA (1994) Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage. Neuron 12:583-595.
40. Roach A, Takahashi N, Pravtcheva D, Ruddle F, Hood L (1985) Chromosomal mapping of mouse myelin basic protein gene and structure and transcription of the partially deleted gene in shiverer mutant mice. Cell 42:149-155.
41. Rosenbluth J (1980) Peripheral myelin in the mouse mutant Shiverer. J Comp Neurol 193:729-739.
42. Schneider A, Montague P, Griffiths I, Fanarraga M, Kennedy P, Brophy P, Nave KA (1992) Uncoupling of hypomyelination and glial cell death by a mutation in the proteotipid protein gene. Nature 358:758-761.
43. Schulte S, Stoffel W (1993) Ceramide UDPgalactosyltransferase from myelinating rat brain: purification, cloning, and expression. Proc Natl Acad Sci USA 90:10265-10269.
44. Stoffel W, Hillen H, Giersiefen H (1984) Structure and molecular arrangement of proteolipid protein of central nervous system myelin. Proc Natl Acad Sci USA 81:5012-5016.
45. Stoffel W, Boison D, Bussow H (1997) Functional analysis in vivo of the double mutant mouse deficient in both proteolipid protein (PLP) and myelin basic protein (MBP) in the central nervous system. Cell Tissue Res 289:195-206.
46. Suzuki K, Poduslo SE, Norton WT (1967) Gangliosides in the myelin fraction of developing rats. Biochim Biophys Acta 144:375-381.
47. Towbin H, Staehelin T, Gordon J (1979) Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications. Proc Natl Acad Sci USA 76:4350-4354.
48. Trapp BD (1990) Myelin-associated glycoprotein. Location and potential functions. Ann NY Acad Sci 605:29-43.
49. Tropak MB, Johnson PW, Dunn RJ, Roder JC (1988) Differential splicing of MAG transcripts during CNS and PNS development. Brain Res 464:143-155.
50. Wallace JE, Krauter EE, Campbell BA (1980) Motor and reflexive behavior in the aging rat. J Gerontol 35:364-370.
51. Weimbs T, Stoffel W (1992) Proteolipid protein (PLP) of CNS myelin: positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues and implications for the membrane topology of PLP Biochemistry 31:12289-12296.
52. Yim SH, Farrer RG, Hammer JA, Yavin E, Quarles RH (1994) Differentiation of oligodendrocytes cultured from developing rat brain is enhanced by exogenous GM3 ganglioside. J Neurosci Res 38:268-281.
53. Yim SH, Farrer RG, Quarles RH (1995) Expression of glycolipids and myelin-associated glycoprotein during the differentiation of oligodendrocytes: comparison of the CG-4 glial cell line to primary cultures. Dev Neurosci 17:171-180.
54. Yin X, Crawford TO, Griffin JW, Tu P, Lee VM, Li C, Roder J, Trapp BD (1998) Myelin-associated glycoprotein is a myelin signal that modulates the caliber of myelinated axons. J Neurosci 18:1953-1962.