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Endothelial lipopigment as an indicator of α-tocopherol deficiency in two equine neurodegenerative diseases

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Abstract

Two spontaneous neurodegenerative diseases of the horse, equine motor neuron disease (EMND) and equine degenerative myeloencephalopathy (EDM), have been associated with α-tocopherol deficiency, and both were characterized by prominent accumulations of endothelial lipopigment in the small vessels of the spinal cord. These endothelial pigment deposits appear to be reversible. In EMND horses pasture-supplemented for 9 months or more after the progression of weakness and wasting had arrested, there was very little endothelial lipopigment. The origin and the potential effects of these endothelial lipopigment accumulations are discussed.

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References

  1. Banker BQ (1986) The pathology of motor neuron disorders. In: Engel AG, Banker BQ (eds) Myology, part II. McGraw-Hill, New York, pp 2031–2067

    Google Scholar 

  2. Beech J (1987) Equine degenerative myeloencephalopathy. In: Reed SM (ed) Veterinary Clinics of North America. Saunders, Philadelphia, pp 379–383

    Google Scholar 

  3. Bergeron C, Muntasser S, Somerville MJ, Weyer L, Percy ME (1994) Copper/zinc superoxide dismutase mRNA levels are increased in sporadic amyotrophic lateral sclerosis motor neurons. Brain Res 659:272–276

    Google Scholar 

  4. Blythe LL, Craig AM (1992) Equine degenerative myeloencephalopathy. Part 1. Clinical signs and pathogenesis. Comp Cont Educ Pract Vet 14:1215–1221

    Google Scholar 

  5. Blythe LL, Craig AM, Lassen ED, Rowe KE, Appell LH (1991) Serially determined plasma α-tocopherol concentrations and results of the oral vitamin E absorption test in clinically normal horses and in horses with degenerative myeloencephalopathy. Am J Vet Res 52:908–911

    Google Scholar 

  6. Bons DP de (1994) Free radicals and antioxidants in vascular biology: the roles of reaction kinetics, environment and substrate turnover. Q J Med 87:445–453

    Google Scholar 

  7. Bowling AC, Schulz JB, Brown RH, Beal MF (1993) Superoxide dismutase, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis. J Neurochem 61:2322–2325

    Google Scholar 

  8. Cavanagh JB, Nolan CC, Seville MP, Anderson VER, Leigh PN (1993) Routes of excretion of neuronal lysosomal dense bodies after ventricular infusion of leupeptin in the rat: a study using ubiquitin and PGP 9.5 immunocytochemistry. J Neurocytol 22:779–791

    Google Scholar 

  9. Coyle JT, Puttfarcken P (1993) Oxidative stress, glutamate, and neurodegenerative disorders. Science 262:689–695

    Google Scholar 

  10. Cummings JF, de Lahunta A, George C, Fuhrer L, Valentine BA, Cooper BJ, Summers BA, Huxtable CR, Mohammed HO (1990) Equine motor neuron disease; a preliminary report. Cornell Vet 80:357–379

    Google Scholar 

  11. Cummings JF, de Lahunta A, Mohammed HO, Divers TJ, Valentine BA, Summers BA, Cooper BJ, Holmes DF, Casey JW, Trembicki K (1991) Equine motor neuron disease: a new neurologic disorder. Equine Pract 13:15–18

    Google Scholar 

  12. Cummings JF, de Lahunta A, Summers BA, Mohammed HO, Divers TJ, Valentine BA, Trembicki-Graves K (1993) Eosinophilic cytoplasmic inclusions in sporadic equine motor neuron disease: an electron microscopic study. Acta Neuropathol 85: 291–297

    Google Scholar 

  13. Deng H-X, Hentati A, Tainer JA, Iqbal Z, Cayabyab A, Hung W-Y, Getzoff ED, Hu P, Herzfeldt B, Roos RP, Warner C, Deng G, Soriano E, Smyth C, Parge HE, Ahmed A, Roses AD, Hallewell RA, Pericak-Vance MA, Siddique T (1993) Amyotrophic lateral selerosis and structural defects in Cu, Zn superoxide dismutase. Science 261:1047–1051

    Google Scholar 

  14. Dierenfeld ES, Traber MG (1992) Vitamin E status of exotic animals compared with livestock and domestics. In: Packer L, Ruchs J (eds) Vitamin E in health and disease. Marcel Dekker, New York, pp 345–370

    Google Scholar 

  15. Divers TJ, Mohammed HO, Cummings JF, de Lahunta A, Valentine BA, Summers BA, Cooper BJ (1992) Equine motor neuron disease: a new cause of weakness, trembling, and weight loss. Comp Cont Educ Pract Vet 14:1222–1226

    Google Scholar 

  16. Divers TJ, Mohammed HO, Cummings JF, Valentine BA, de Lahunta A, Jackson CA, Summers BA (1994) Equine motor neuron disease: findings in twenty-eight horses and proposal of a pathophysiological mechanism for the disease. Equine Vet J 26:409–415

    Google Scholar 

  17. Fischer VW, Nelson JS (1973) Cerebrovascular changes in tocopherol depleted chicks fed linoleic acid. J Neuropathol Exp Neurol 32:474–483

    Google Scholar 

  18. Gurney ME, Pu H, Chiu AY, Dal C anto MC, Polchow CY, Alexander DD, Caliendo J, Hentati A, Kwon YW, Deng H-X, Chen W, Zhai P, Sufit RA, Siddique T (1994) Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 264:1772–1775

    Google Scholar 

  19. Kinnula VL, Mirza Z, Crapo JD, Whorton AR (1993) Modulation of hydrogen peroxide released from vascular endothelial cells by oxygen. Am J Respir Cell Mol Biol 9:603–609

    Google Scholar 

  20. Koppang N (1979) Neuronal ceroid-lipofuscinosis (Batten disease). In: Andrews EJ, Ward BC, Altman NH (eds) Spontaneous animal models of human disease, vol 2. Academic Press, New York, p 136

    Google Scholar 

  21. Lampert PN, Blumberg JM, Pentschew A (1964) An electron microscopic study of dystrophic axons in the gracile and cuneate nuclei of vitamin E-deficient rats. J Neuropathol Exp Neurol 23:60–77

    Google Scholar 

  22. Liu SK, Dolansek EP, Adams CR, Tappe JP (1983) Myelopathy and vitamin E deficiency in six Mongolian wild horses. J Am Vet Med Assoc 183:1266–1268

    Google Scholar 

  23. Mayhew IG (1989) Large animal neurology, a handbook for veterinary clinicians. Lea and Febiger, Philadelphia, pp 294–296

    Google Scholar 

  24. Mayhew IG, de Lahunta A, Whitlock RH, Geary JC (1977) Equine degenerative myeloencephalopathy. J Am Vet Med Assoc 170:195–201

    Google Scholar 

  25. Mayhew IG, de Lahunta A, Whitlock RH, Krook L, Tasker JB (1978) Spinal cord disease in the horse. Cornell Vet 68 [Suppl 8]:1–207

    Google Scholar 

  26. Mayhew IG, Brown CM, Stowe HD, Trapp AL, Derkson FJ, Clement SF (1987) Equine degenerative myeloencephalopathy: a vitamin E deficiency that may be familial. J Vet Intern Med 1:45–49

    Google Scholar 

  27. Mohammed HO, Cummings JF, Divers TJ, Valentine B, de Lahunta A, Summers B, Farrow BR, Trembicki-Graves K, Mauskopf A (1993) Risk factors associated with equine motor neuron disease: a possible model for human. Neurology 43: 966–971

    Google Scholar 

  28. Montali RJ, Bush M, Sauer RM, Gray CW, Xanten WA (1974) Spinal ataxia in zebra. Comparison with wobbler syndrome of horses. Vet Pathol 11:68–78

    Google Scholar 

  29. Muller DPR, Goss-Sampson MA (1990) Neurochemical, neurophysiological, and neuropathological studies in vitamin E deficiency. Crit Rev Neurobiol 5:239–263

    Google Scholar 

  30. Nelson JS (1983) Neuropathological studies of chronic vitamin E deficiency in mammals including humans. Ciba Found Symp 101:92–105

    Google Scholar 

  31. Nelson JS, Fitch CD, Fischer VW, Brown GO, Chou AC (1981) Progressive neuropathologic lesions in vitamin E deficient rhesus monkeys. J Neuropathol Exp Neurol 40:166–186

    Google Scholar 

  32. Oldendorf WH, Cornford ME, Brown WJ (1977) The large apparent work capacity of the blood-brain barrier. A study of the mitochondrial content of capillary endothelial cells in brain and other tissues of the rat. Ann Neurol 1:409–417

    Google Scholar 

  33. Palmer DN, Fearnley IM, Walker JE, Hall NA, Lake BD, Wolfe LS, Haltia M, Martinus RD, Jolly RD (1992) Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease). Am J Med Genet 42:561–567

    Google Scholar 

  34. Pearson K (1913) On the probable error of a coefficient of correlation as found from a fourfold table. Biometrika 9:22–27

    Google Scholar 

  35. Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, Donaldson D, Goto J, O'Regan JP, Deng H-X, Rahmani Z, Krizus A, McKenna-Yasek D, Cayabyab A, Gaston SM, Berger R, Tanzi RE, Halperin JJ, Herzfeldt B, Bergh R van den, Hung W-Y, Bird T, Deng G, Mulder DW, Smyth C, Laing NG, Soriano E, Pericak-Vance MA, Haines J, Rouleau GA, Gusella JS, Horvitz HR, Brown RH Jr (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362:59–62

    Google Scholar 

  36. Schochet SS (1972) Neuronal inclusions. In: Bourne GH (ed) The structure and function of nervous tissue, vol 4. Academic Press, New York, pp 129–177

    Google Scholar 

  37. Siddique T, Figlewicz DA, Pericak-Vance MA, Haines JL, Rouleau G, Jeffers AJ, Sapp P, Hung W-Y, Bebout J, McKenna-Yasek D, Deng G, Horvitz HR, Gusella JF, Brown RH Jr, Roses AD (1991) Linkage of a gene causing familial amyotrophic lateral sclerosis to chromosome 21 and evidence of genetic-locus heterogencity. N Engl J Med 324:1381–1384

    Google Scholar 

  38. Summers BA, Cummings JF, de Lahunta A (1995) Veterinary neuropathology. Mosby, St Louis, pp 309–312

    Google Scholar 

  39. Thomas PK, Cooper JM, King RHM, Workman JM, Schapira AHV, Goss-Sampson MA, Muller DPR (1993) Myopathy in vitamin E deficient rats: muscle fibre necrosis associated with disturbances of mitochondrial function. J Anat 183:451–461

    Google Scholar 

  40. Valentine BA, de Lahunta A, George C, Summers BA, Cummings JF, Divers TJ, Mohammed HO (1994) Acquired equine motor neuron disease. Vet Pathol 31:130–138

    Google Scholar 

  41. Wilcoxon F (1945) Individual comparisons by ranking methods. Biometrics 1:80–83

    Google Scholar 

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Cummings, J.F., de Lahunta, A., Mohammed, H.O. et al. Endothelial lipopigment as an indicator of α-tocopherol deficiency in two equine neurodegenerative diseases. Acta Neuropathol 90, 266–272 (1995). https://doi.org/10.1007/BF00296510

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  • DOI: https://doi.org/10.1007/BF00296510

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