Increases in Fragmented Glial Fibrillary Acidic Protein Levels in the Spinal Cords of Patients with Amyotrophic Lateral Sclerosis

Overview

Journal Neurochem Res

Specialties Chemistry
Neurology

Date 1998 Feb 25

PMID 9475511

Citations 15

Authors

K Fujita

T Kato

M Yamauchi

M Ando

M Honda

Y Nagata

Affiliations

Soon will be listed here.

Abstract

Using one-dimensional polyacrylamide gel electrophoresis, we analyzed protein fractions extracted from the spinal cords of patients with amyotrophic lateral sclerosis (ALS). Several protein bands with molecular weights of 35-55 kDa were stained with Coomassie brilliant blue much more intensely in the ALS than in the non-ALS spinal cord. Glial fibrillary acidic protein (GFAP) immunoreactivity showed a significant decrease of 50 and 45 kDa band and increase in fragmented 36 and 37 kDa bands, which represented GFAP fragments devoid of 59 and 40 residues from the N-terminal, respectively, as determined by protein sequence analysis. Immunohistochemical examination of ALS spinal cord transections demonstrated increased GFAP-stained astrocytes in the shrunken ventral horn with massive degeneration of motoneurons. These results will provide new insight into the possible role of astrocytes in the pathophysiology and/or pathogenesis of ALS.

Citing Articles

Development of Tissue-Engineered Model of Fibrotic Scarring after Spinal Cord Injury to Study Astrocyte Activation and Neurite Outgrowth In Vitro.

Ala-Kokko N, Baek I, Song Y ACS Biomater Sci Eng. 2024; 10(10):6545-6557.

PMID: 39259933 PMC: 11480936. DOI: 10.1021/acsbiomaterials.4c01100.

Astrocyte-Neuron Interactions Contributing to Amyotrophic Lateral Sclerosis Progression.

Jensen B Adv Neurobiol. 2024; 39:285-318.

PMID: 39190080 DOI: 10.1007/978-3-031-64839-7_12.

GFAP as Astrocyte-Derived Extracellular Vesicle Cargo in Acute Ischemic Stroke Patients-A Pilot Study.

Forro T, Manu D, Bajenaru O, Balasa R Int J Mol Sci. 2024; 25(11).

PMID: 38891912 PMC: 11172178. DOI: 10.3390/ijms25115726.

Microglia and Astrocytes in Amyotrophic Lateral Sclerosis: Disease-Associated States, Pathological Roles, and Therapeutic Potential.

You J, Youssef M, Santos J, Lee J, Park J Biology (Basel). 2023; 12(10).

PMID: 37887017 PMC: 10603852. DOI: 10.3390/biology12101307.

Characterization of Calpain and Caspase-6-Generated Glial Fibrillary Acidic Protein Breakdown Products Following Traumatic Brain Injury and Astroglial Cell Injury.

Yang Z, Arja R, Zhu T, Sarkis G, Patterson R, Romo P Int J Mol Sci. 2022; 23(16).

PMID: 36012232 PMC: 9409281. DOI: 10.3390/ijms23168960.

References

Troost D, Sillevis Smitt P, de Jong J, Swaab D . Neurofilament and glial alterations in the cerebral cortex in amyotrophic lateral sclerosis. Acta Neuropathol. 1992; 84(6):664-73. DOI: 10.1007/BF00227744. View

Eng L, Rubinstein L . Contribution of immunohistochemistry to diagnostic problems of human cerebral tumors. J Histochem Cytochem. 1978; 26(7):513-22. DOI: 10.1177/26.7.357640. View

Rosengren L, Lycke J, Andersen O . Glial fibrillary acidic protein in CSF of multiple sclerosis patients: relation to neurological deficit. J Neurol Sci. 1995; 133(1-2):61-5. DOI: 10.1016/0022-510x(95)00152-r. View

Stone G, Hammerschlag R . Differential effects of cobalt on the initiation of fast axonal transport. Cell Mol Neurobiol. 1981; 1(1):3-17. PMC: 11572842. DOI: 10.1007/BF00736036. View

Tetzlaff W, Graeber M, Bisby M, Kreutzberg G . Increased glial fibrillary acidic protein synthesis in astrocytes during retrograde reaction of the rat facial nucleus. Glia. 1988; 1(1):90-5. DOI: 10.1002/glia.440010110. View

Murachi T . Intracellular regulatory system involving calpain and calpastatin. Biochem Int. 1989; 18(2):263-94. View

Bigbee J, Bigner D, Pegram C, Eng L . Study of glial fibrillary acidic protein in a human glioma cell line grown in culture and as a solid tumor. J Neurochem. 1983; 40(2):460-7. DOI: 10.1111/j.1471-4159.1983.tb11305.x. View

Delacourte A . General and dramatic glial reaction in Alzheimer brains. Neurology. 1990; 40(1):33-7. DOI: 10.1212/wnl.40.1.33. View

Rosengren L, Karlsson J, Karlsson J, Persson L, Wikkelso C . Patients with amyotrophic lateral sclerosis and other neurodegenerative diseases have increased levels of neurofilament protein in CSF. J Neurochem. 1996; 67(5):2013-8. DOI: 10.1046/j.1471-4159.1996.67052013.x. View

10.

Nagata Y, Okuya M, Watanabe R, Honda M . Regional distribution of cholinergic neurons in human spinal cord transections in the patients with and without motor neuron disease. Brain Res. 1982; 244(2):223-9. DOI: 10.1016/0006-8993(82)90081-6. View

11.

Matsui T, Fujimura Y, Nishida S, Titani K . Human plasma alpha 2-macroglobulin and von Willebrand factor possess covalently linked ABO(H) blood group antigens in subjects with corresponding ABO phenotype. Blood. 1993; 82(2):663-8. View

12.

Benuck M, Banay-Schwartz M, Deguzman T, Lajtha A . Changes in brain protease activity in aging. J Neurochem. 1996; 67(5):2019-29. DOI: 10.1046/j.1471-4159.1996.67052019.x. View

13.

Gomi H, Yokoyama T, Fujimoto K, Ikeda T, Katoh A, Itoh T . Mice devoid of the glial fibrillary acidic protein develop normally and are susceptible to scrapie prions. Neuron. 1995; 14(1):29-41. DOI: 10.1016/0896-6273(95)90238-4. View

14.

Nakamura Y, Takeda M, Aimoto S, Hojo H, Takao T, Shimonishi Y . Assembly regulatory domain of glial fibrillary acidic protein. A single phosphorylation diminishes its assembly-accelerating property. J Biol Chem. 1992; 267(32):23269-74. View

15.

Liedtke W, Edelmann W, Bieri P, Chiu F, Cowan N, Kucherlapati R . GFAP is necessary for the integrity of CNS white matter architecture and long-term maintenance of myelination. Neuron. 1996; 17(4):607-15. DOI: 10.1016/s0896-6273(00)80194-4. View

16.

Fujita K, Yamauchi M, Shibayama K, Ando M, Honda M, Nagata Y . Decreased cytochrome c oxidase activity but unchanged superoxide dismutase and glutathione peroxidase activities in the spinal cords of patients with amyotrophic lateral sclerosis. J Neurosci Res. 1996; 45(3):276-81. DOI: 10.1002/(SICI)1097-4547(19960801)45:3<276::AID-JNR9>3.0.CO;2-A. View

17.

Guesdon J, Ternynck T, Avrameas S . The use of avidin-biotin interaction in immunoenzymatic techniques. J Histochem Cytochem. 1979; 27(8):1131-9. DOI: 10.1177/27.8.90074. View

18.

Fujita K, Yamauchi M, Matsui T, Titani K, Takahashi H, Kato T . Increase of glial fibrillary acidic protein fragments in the spinal cord of motor neuron degeneration mutant mouse. Brain Res. 1998; 785(1):31-40. DOI: 10.1016/s0006-8993(97)00612-4. View

19.

Caroscio J, Mulvihill M, Sterling R, Abrams B . Amyotrophic lateral sclerosis. Its natural history. Neurol Clin. 1987; 5(1):1-8. View

20.

Fujita K, Ando M, Yamauchi M, Nagata Y, Honda M . Alteration of transglutaminase activity in rat and human spinal cord after neuronal degeneration. Neurochem Res. 1995; 20(10):1195-201. DOI: 10.1007/BF00995383. View