Immunoexpression of gemins 2 and 4 in the rat spinal cord. Is the SMN complex a new target in investigations of sporadic amyotrophic lateral sclerosis pathogenesis?

Folia Neuropathologica

Volumn 50, Issue 4, 2012, Pages 390-396

  Rafałowska, Janina ^a   Sulejczak, Dorota ^a   Gadamski, Roman ^a   Dziewulska, Dorota ^a,b  

^a MOSSAKOWSKI MEDICAL RESEARCH CENTRE   (Poland)

^b MEDICAL UNIVERSITY OF WARSAW   (Poland)

Author keywords

ALS; Gemin; Rat; SMN

Indexed keywords

GEMIN 2; GEMIN 4; NUCLEAR PROTEIN; SURVIVAL MOTOR NEURON PROTEIN; UNCLASSIFIED DRUG;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ANIMAL TISSUE; ANTIBODY LABELING; ARTICLE; CONTROLLED STUDY; IMMUNOFLUORESCENCE TEST; IMMUNOHISTOCHEMISTRY; IMMUNOREACTIVITY; LIFESPAN; MALE; NONHUMAN; PATHOGENESIS; PERINATAL PERIOD; PROTEIN ANALYSIS; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN LOCALIZATION; RAT; SPINAL CORD; SPINAL CORD NERVE CELL;

EID: 84873379912     PISSN: 16414640     EISSN: 1509572X     Source Type: Journal    
DOI: 10.5114/fn.2012.32373     Document Type: Article

References (24)

1. Cauchi RJ, Sanchez-Pulido L, Liu JL. Drosophila SMN complex proteins Gemin2, Gemin3, and Gemin5 are components of U bodies. Exp Cell Res 2010; 316: 2354-2364.
2. Charroux B, Pellizzoni L, Perkinson RA, Yong J, Shevchenko A, Mann M, Dreyfuss G. Gemin 4: a novel component of the SMN complex that is found in both gems and nuclei. J Cell Biol 2000; 148: 1177-1186.
3. Corcia P, Camu W, Halimi JM, Vourc'h P, Antar C, Vedrine S, Giraudeau B, de Toffol B, Andres CR; French ALS Study Group. SMN1 gene, but not SMN2, is a risk factor for sporadic ALS. Neurology 2006; 67: 1147-1150.
4. Corcia P, Camu W, Praline J, Gordon PH, Vourch P, Andres C. The importance of the SMN genes in the genetics of sporadic ALS. Amyotroph Lateral Scler 2009; 10: 436-440.
5. Gertz B, Wong M, Martin LJ. Nuclear localization of human SOD1 and mutant SOD1-specific disruption of survival motor neuron protein complex in transgenic amyotrophic lateral sclerosis mice. J Neuropathol Exp Neurol 2012; 71: 162-177.
6. Giavazzi A, Setola V, Simonati A, Battaglia G. Neuronal-specific roles of the survival motor neuron protein: evidence from survival motor neuron expression patterns in the developing human central nervous system. J Neuropathol Exp Neurol 2006; 65: 267-377.
7. th ed. Oxford University Press, Oxford 2008.
8. Hao le T, Fuller HR, Lam le T, Le TT, Burghes AH, Morris GE. Absence of gemin5 from SMN complexes in nuclear Cajal bodies. BMC Cell Biol 2007; 18: 8-28.
9. Kolb SJ, Battle DJ, Dreyfuss G. Molecular Functions of the SMN Complex. J Child Neurol 2007; 22: 990-994.
10. La Bella V, Cisterni C, Salaün D, Pettman B. Survival motor neuron (SMN) protein in rat is expressed as different molecular forms and is developmentally regulated. Eur J Neurosci 1998; 10: 2913-2923.
11. Lorson MA, Dickson AM, Shaw DJ, Todd AG, Young EC, Morse R, Wolstencroft C, Lorson CL, Young PJ. Identification and characterisation of a nuclear localisation signal in the SMN associated protein, Gemin4. Biochem Biophys Res Commun 2008; 375: 33-37.
12. Lührmann R, Kastner B, Bach M. Structure of spliceosomal snRNPs and their role in pre-mRNA splicing. Biochim Biophys Acta 1990; 1087: 265-292.
13. Lutz CM, Kariya S, Patruni S, Osborne MA, Liu D, Henderson CE, Li DK, Pellizzoni L, Rojas J, Valenzuela DM, Murphy AJ, Winberg ML, Monani UR. Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy. J Clin Invest 2011; 121: 3029-3041.
14. Naganska E, Matyja E. Amyotrophic lateral sclerosis - looking for pathogenesis and effective therapy. Folia Neuropathol 2011; 49: 1-13.
15. Naganska E, Taraszewska A, Matyja E, Grieb P, Rafalowska J. Neu-roprotective effect of erythropoietin in amyotrophic lateral sclerosis (ALS) model in vitro. Ultrastructural study. Folia Neuropathol 2010; 48: 35-44.
16. Ogawa C, Usui K, Aoki M, Ito F, Itoh M, Kai C, Kanamori-Katayama M, Hayashizaki Y, Suzuki H. Gemin 2 plays an important role in stabilizing the survival of motor neuron complex. J Biol Chem 2007; 282: 11122-11134.
17. Pellizzoni L, Charroux B, Dreyfuss G. SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins. Proc Natl Acad Sci USA 1999; 96: 11167-11172.
18. Piao Y, Hashimoto T, Takahama S, Kakita A, Komori T, Morita T, Taka-hashi H, Mizutani T, Oyanagi K. Survival motor neuron (SMN) protein in the spinal anterior horn cells of patients with sporadic amyotrophic lateral sclerosis. Brain Res 2011; 1372: 152-159.
19. Rafalowska J, Sulejczak D, Gadamski R, Dziewulska D. Survival motor neuron (SMN) - a motor neuron insurance for a whole lifespan? Folia Neuropathol 2011; 49: 301-310.
20. Turner BJ, Parkinson NJ, Davies KE, Talbot K. Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model. Neurobiol Dis 2009; 34: 511-517.
21. Veldink JH, Kalmijn S, Van der Hout AH, Lemmink HH, Groeneveld GJ, Lummen C, Scheffer H, Wokke JH, Van der Berg LH. SMN genotypes producing less SMN protein increase susceptibility to and severity of sporadic ALS. Neurology 2005; 65: 820-825.
22. van Blitterswijk M, Landers JE. RNA processing pathways in amyotrophic lateral sclerosis. Neurogenetics 2010; 11: 275-290.
23. Zhang H, Xing L, Rossoll W, Wichterle H, Singer RH, Bassell GJ. Multiprotein complexes of the survival of motor neuron protein SMN with Gemins traffic to neuronal processes and growth cones of motor neurons. J Neurosci 2006; 26: 8622-8632.
24. Zou T, Ilangovan R, Yu F, Xu Z, Zhou J. SMN protects cells against mutant SOD1 toxicity by increasing chaperone activity. Biochem Biophys Res Commun 2007; 364: 850-855.