Expression of myelin proteins in the adult heterozygous Trembler mouse

Acta Neuropathologica

Volumn 98, Issue 3, 1999, Pages 281-287

  Vallat, J M ^a   Sindou, P ^a   Garbay, B ^b   Preux, P M ^a   Anani, T ^a   Richard, L ^a   Diot, M ^a  

^a DUPUYTREN UNIVERSITY HOSPITAL   (France)

^b University Victor Segalen   (France)

Author keywords

Genetic neuropathies; Myelin proteins; Peripheral nerve; Trembler mouse

Indexed keywords

ASPARTIC ACID; GLYCINE; MYELIN ASSOCIATED GLYCOPROTEIN; MYELIN BASIC PROTEIN; MYELIN PROTEIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL MATURATION; CELL ULTRASTRUCTURE; CONTROLLED STUDY; GENE MUTATION; HEREDITARY MOTOR SENSORY NEUROPATHY; IMMUNOCYTOCHEMISTRY; MOUSE; MYELIN SHEATH; MYELINATED NERVE; MYELINATION; NERVE BIOPSY; NERVE FIBER; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; SCHWANN CELL; SCIATIC NERVE;

AGE FACTORS; ANIMALS; CHARCOT-MARIE-TOOTH DISEASE; DISEASE MODELS, ANIMAL; HETEROZYGOTE; MICE; MICE, NEUROLOGIC MUTANTS; MICROSCOPY, IMMUNOELECTRON; MYELIN P0 PROTEIN; MYELIN PROTEINS; MYELIN SHEATH; MYELIN-ASSOCIATED GLYCOPROTEIN; NERVE FIBERS, MYELINATED; POINT MUTATION; SCIATIC NERVE;

EID: 0032840812     PISSN: 00016322     EISSN: None     Source Type: Journal    
DOI: 10.1007/s004010051081     Document Type: Article

References (18)

1. Ayers MM, Anderson RMcD (1973) Onion bulb neuropathy in the Trembler mouse: a model of hypertrophic interstitial neuropathy (Dejerine-Sottas) in man. Acta Neuropathol (Berl) 25: 54-70
2. Ayers MM, Anderson RMcD (1976) Development of onion bulb neuropathy in the Trembler mouse; morphometric study. Acta Neuropathol (Berl) 36: 137-152
3. Bascles L, Bonnet J, Garbay B (1992) Expression of the PMP22 gene in Trembler mutant mice: comparison with the other myelin protein genes. Dev Neurosci 14: 336-341
4. D'Urso D, Schmalenbach C, Zoild G, Prior R, Müller HW (1997) Studies on the effects of altered PMP22 expression during myelination in vitro. J Neurosci Res 48: 31-42
5. Garbay B, Domec C, Fournier M, Bonnet J (1989) Developmental expression of the P0 glycoprotein and basic protein mRNAs in normal and Trembler mutant mice. J Neurochem 53:907-911
6. Garbay B, Bonnet J (1992) P0 protein in normal, Trembler heterozygous/homozygous mice during active PNS myelination. NeuroReport 3: 594-596
7. Inuzuka T, Quarles RH, Heath J, Trapp BD (1985) Myelinassociated glycoprotein and other proteins in Trembler mice. J Neurochem 44: 793-797
8. Jacque C; Delassale A, Raoul M, Baumann N (1983) Myelin basic protein deposition in the optic and sciatic nerves of dysmyelinating mutants quaking, jimpy, Trembler, mld and shiverer during development. J Neurochem 41: 1335-1340
9. Kirschner DA, Szumowski K, Gabreëls-Festen AAWM, Hoogendijk JE, Bolhuis PA (1996) Inherited demyelinating peripheral neuropathies: relating myelin packing abnormalities to P0 molecular defects. J Neurosci Res 46: 502-508
10. Low PA (1976) Hereditary hypertrophic neuropathy in the Trembler mouse. Part 2. Histopathological studies: electron microscopy. J Neurol Sci 30: 343-368
11. Navon R, Seifried B, Shoham Gal-On N, Sadeh M (1996) A new mutation affecting the fourth transmembrane domain of PMP22 results in severe, de novo Charcot-Marie-Tooth disease. Hum Genet 97: 685-687
12. Notterpek L, Shooter EM, Snipes GJ (1997) Upregulation of the endosomal-lysosomal pathway in the Trembler-J neuropathy. J Neurosci 17: 4190-4200
13. Robertson AM, King RHM, Muddle JR, Thomas PK (1997) Abnormal Schwann cell/axon interactions in the Trembler-J mouse. J Anat 190: 423-432
14. Snipes GJ, Suter U (1995) Molecular anatomy and genetics of myelin proteins in the peripheral nervous system. J Anat 186: 483-494
15. Suh JG, Ichihara N, Saigoh K, Nakabayashi O, Yamanishi T, Tanaka K, Wada K, Kikuchi T (1997) An in-frame deletion in peripheral myelin protein-22 gene causes hypomyelination and cell death of the Schwann cells in the new Trembler mutant mice. Neurosci 79: 735-744
16. Tokuyasu KT (1973) A technique for ultracryotomy of cell suspensions and tissues. J Cell Biol 57: 551-565
17. Trapp BD, Quarles RH (1982) Presence of myelin-associated glycoprotein correlates with alterations in the periodicity of peripheral myelin. J Cell Biol 92: 877-88
18. Vallat JM, Sindou P, Preux PM, Tabaraud F, Milor AM, Couratier P, LeGuern E, Brice A (1996) Ultrastructural PMP22 expression in inherited demyelinating neuropathies. Ann Neurol 36: 813-817