Dp71, Utrophin and Beta-dystroglycan Expression and Distribution in PC12/L6 Cell Cocultures

Overview

Journal Neuroreport

Specialty Neurology

Date 2007 Oct 9

PMID 17921863

Citations 1

Authors

Ramses Ilarraza-Lomeli

Bulmaro Cisneros-Vega

Maria de Lourdes Cervantes-Gomez

Dominique Mornet

Cecilia Montanez

Affiliations

Soon will be listed here.

Abstract

Function of dystrophin Dp71 isoforms is unknown but seems related to neurite outgrowth and synapse formation. To evaluate Dp71 role in myoneural synapses, we established a coculture model using PC12 cells and L6 myotubes and analyzed expression and localization of Dp71 and related proteins, utrophin and beta-dystroglycan, in PC12 cells. Confocal microscopy showed Dp71d isoform in PC12 nuclei, golgi-complex-like and endoplasmic reticulum-like structures, whereas Dp71ab concentrates at neurite tips and cytoplasm, colocalizing with beta-dystroglycan, utrophin, synaptophysin and acetylcholine receptors. Evidences suggest that Dp71ab isoform, unlike Dp71d, may take part in neurite-related processes. This is the first work on Dp and members of Dp-associated protein complex roles in a cell-line based coculturing system, which may be useful in determining Dp71 isoforms associations.

Citing Articles

Human Dystrophin Dp71ab Enhances the Proliferation of Myoblasts Across Species But Not Human Nonmyoblast Cells.

Farea M, Maeta K, Nishio H, Matsuo M Front Cell Dev Biol. 2022; 10:877612.

PMID: 35547811 PMC: 9081641. DOI: 10.3389/fcell.2022.877612.

References

Fabbrizio E, Nudel U, Hugon G, Robert A, Pons F, Mornet D . Characterization and localization of a 77 kDa protein related to the dystrophin gene family. Biochem J. 1994; 299 ( Pt 2):359-65. PMC: 1138280. DOI: 10.1042/bj2990359. View

Apel E, Glass D, Moscoso L, Yancopoulos G, Sanes J . Rapsyn is required for MuSK signaling and recruits synaptic components to a MuSK-containing scaffold. Neuron. 1997; 18(4):623-35. DOI: 10.1016/s0896-6273(00)80303-7. View

Prives J, Bar-Sagi D . Effect of tunicamycin, an inhibitor of protein glycosylation, on the biological properties of acetylcholine receptor in cultured muscle cells. J Biol Chem. 1983; 258(3):1775-80. View

Hoffman E, Brown Jr R, Kunkel L . Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell. 1987; 51(6):919-28. DOI: 10.1016/0092-8674(87)90579-4. View

Nagata K, Huang C, Song J, Narahashi T . Lead modulation of the neuronal nicotinic acetylcholine receptor in PC12 cells. Brain Res. 1997; 754(1-2):21-7. DOI: 10.1016/s0006-8993(97)00037-1. View

Tommasi di Vignano A, Di Zenzo G, Sudol M, Cesareni G, Dente L . Contribution of the different modules in the utrophin carboxy-terminal region to the formation and regulation of the DAP complex. FEBS Lett. 2000; 471(2-3):229-34. DOI: 10.1016/s0014-5793(00)01400-9. View

Durbeej M, Campbell K . Muscular dystrophies involving the dystrophin-glycoprotein complex: an overview of current mouse models. Curr Opin Genet Dev. 2002; 12(3):349-61. DOI: 10.1016/s0959-437x(02)00309-x. View

Laska D, Williams P, White S, Thompson C, Hoover D . In vitro correlation of ultrastructural morphology and creatine phosphokinase release in L6 skeletal muscle cells after exposure to parenteral antibiotics. In Vitro Cell Dev Biol. 1990; 26(4):393-8. DOI: 10.1007/BF02623831. View

Ervasti J . Dystrophin, its interactions with other proteins, and implications for muscular dystrophy. Biochim Biophys Acta. 2006; 1772(2):108-17. DOI: 10.1016/j.bbadis.2006.05.010. View

10.

Jung D, Yang B, Meyer J, Chamberlain J, Campbell K . Identification and characterization of the dystrophin anchoring site on beta-dystroglycan. J Biol Chem. 1995; 270(45):27305-10. DOI: 10.1074/jbc.270.45.27305. View

11.

Wiedenmann B, REHM H, Knierim M, Becker C . Fractionation of synaptophysin-containing vesicles from rat brain and cultured PC12 pheochromocytoma cells. FEBS Lett. 1988; 240(1-2):71-7. DOI: 10.1016/0014-5793(88)80342-9. View

12.

Tokarz S, Duncan N, Rash S, Sadeghi A, Dewan A, Pillers D . Redefinition of dystrophin isoform distribution in mouse tissue by RT-PCR implies role in nonmuscle manifestations of duchenne muscular dystrophy. Mol Genet Metab. 1999; 65(4):272-81. DOI: 10.1006/mgme.1998.2763. View

13.

Gramolini A, Jasmin B . Expression of the utrophin gene during myogenic differentiation. Nucleic Acids Res. 1999; 27(17):3603-9. PMC: 148607. DOI: 10.1093/nar/27.17.3603. View

14.

Ren X, Cheng S, Treuil M, Mukherjee J, Rao J, Braunewell K . Structural determinants of alpha4beta2 nicotinic acetylcholine receptor trafficking. J Neurosci. 2005; 25(28):6676-86. PMC: 6725434. DOI: 10.1523/JNEUROSCI.1079-05.2005. View

15.

Adams M, Butler M, Dwyer T, Peters M, Murnane A, Froehner S . Two forms of mouse syntrophin, a 58 kd dystrophin-associated protein, differ in primary structure and tissue distribution. Neuron. 1993; 11(3):531-40. DOI: 10.1016/0896-6273(93)90157-m. View

16.

Daniels M, Lowe B, Shah S, Ma J, Samuelsson S, Lugo B . Rodent nerve-muscle cell culture system for studies of neuromuscular junction development: refinements and applications. Microsc Res Tech. 2000; 49(1):26-37. DOI: 10.1002/(SICI)1097-0029(20000401)49:1<26::AID-JEMT4>3.0.CO;2-8. View

17.

Kramarcy N, Vidal A, Froehner S, Sealock R . Association of utrophin and multiple dystrophin short forms with the mammalian M(r) 58,000 dystrophin-associated protein (syntrophin). J Biol Chem. 1994; 269(4):2870-6. View

18.

Cisneros B, Rendon A, Genty V, Aranda G, Marquez F, Mornet D . Expression of dystrophin Dp71 during PC12 cell differentiation. Neurosci Lett. 1996; 213(2):107-10. DOI: 10.1016/0304-3940(96)12863-9. View

19.

Wilson J, Putt W, Jimenez C, Edwards Y . Up71 and up140, two novel transcripts of utrophin that are homologues of short forms of dystrophin. Hum Mol Genet. 1999; 8(7):1271-8. DOI: 10.1093/hmg/8.7.1271. View

20.

Tinsley J, Blake D, Roche A, Fairbrother U, Riss J, Byth B . Primary structure of dystrophin-related protein. Nature. 1992; 360(6404):591-3. DOI: 10.1038/360591a0. View