Effect of Propionic and Methylmalonic Acids on the High Molecular Weight Neurofilament Subunit (NF-H) in Rat Cerebral Cortex

作者: L. Vivian , F. Dall Bello Pessutto , L. M. Vieira de Almeida , S. de Oliveira Loureiro , P. de Lima Pelaez

DOI: 10.1023/A:1021699314007

关键词: ChemistryPhosphorylationNeurofilamentMethylmalonic acidDephosphorylationMolecular biologyCytoskeletonCerebral cortexSpecific antibodyBiochemistryProtein subunit

摘要: Propionic and methylmalonic acidemias are inherited neurometabolic disorders biochemically characterized by tissue accumulation of propionic (PA) (MMA) acids, respectively. Neurofilaments (NF) important cytoskeletal proteins phosphorylation/dephosphorylation NF is to stabilize the cytoskeleton. We investigated effects PA MMA on high molecular weight neurofilament subunit associated with fraction rat cerebral cortex along development. Cortical slices from 9- 60-day-old rats were incubated 2.5 mM or MMA. The was extracted immunoreactivity for phosphorylated total NF-H analyzed immunoblotting using specific antibodies. Results showed that treatment acids induced an increased Triton-insoluble in up 17-day-old rats. Furthermore, treatments significantly amount 12-day-old These findings indicate alter dynamic regulation assembly fraction.

参考文章(41)
R. A. Chalmers, A. M. Lawson, Disorders of propionate and methylmalonate metabolism Springer, Dordrecht. pp. 296- 331 ,(1982) , 10.1007/978-94-009-5778-7_11
S. Hisanaga, M. Kusubata, E. Okumura, T. Kishimoto, Phosphorylation of neurofilament H subunit at the tail domain by CDC2 kinase dissociates the association to microtubules. Journal of Biological Chemistry. ,vol. 266, pp. 21798- 21803 ,(1991) , 10.1016/S0021-9258(18)54707-4
A. Lewis Farr, Oliver H. Lowry, Rose J. Randall, Nira J. Rosebrough, Protein Measurement with the Folin Phenol Reagent Journal of Biological Chemistry. ,vol. 193, pp. 265- 275 ,(1951)
Michael J. Strong, Wendy L. Strong, Howard Jaffe, Bearnd Traggert, Maggie M. Sopper, Harish C. Pant, Phosphorylation state of the native high-molecular-weight neurofilament subunit protein from cervical spinal cord in sporadic amyotrophic lateral sclerosis. Journal of Neurochemistry. ,vol. 76, pp. 1315- 1325 ,(2001) , 10.1046/J.1471-4159.2001.00094.X
P T Ozand, J Brismar, CT and MR of the brain in disorders of the propionate and methylmalonate metabolism. American Journal of Neuroradiology. ,vol. 15, pp. 1459- 1473 ,(1994)
Michael K. Lee, Don W. Cleveland, Neurofilament function and dysfunction: involvement in axonal growth and neuronal disease Current Opinion in Cell Biology. ,vol. 6, pp. 34- 40 ,(1994) , 10.1016/0955-0674(94)90113-9
M. L. Schmidt, John A. Martin, Virginia M.-Y. Lee, John Q. Trojanowski, Convergence of Lewy bodies and neurofibrillary tangles in amygdala neurons of Alzheimer’s disease and Lewy body disorders Acta Neuropathologica. ,vol. 91, pp. 475- 481 ,(1996) , 10.1007/S004010050454
G. F. Hoffmann, W. Meier-Augenstein, S. St�ckler, R. Surtees, D. Rating, W. L. Nyhan, Physiology and pathophysiology of organic acids in cerebrospinal fluid Journal of Inherited Metabolic Disease. ,vol. 16, pp. 648- 669 ,(1993) , 10.1007/BF00711898
N Hirokawa, M A Glicksman, M B Willard, Organization of mammalian neurofilament polypeptides within the neuronal cytoskeleton Journal of Cell Biology. ,vol. 98, pp. 1523- 1536 ,(1984) , 10.1083/JCB.98.4.1523