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CRYSTAL-LIKE INCLUSIONS IN HEART MITOCHONDRIA
620 recorded after a few weeks of classical treatment. 2 of our patients received the treatment by levodopa for four months at recommended optimum doses; both had a significant aggravation of their lesions. The results of Barbeau (personal communication) are quite different from ours, and we agree with him that double-blind trials are required to establish the eventual place of levodopa in the treatment of psoriasis. Institute of Medicine,
DEVOITILLE.
University of Liège, Belgium
JEAN-MICHEL
Department of Dermatology, Hôpital de Bavière, University of Liège, Belgium
MICHEL-F. DE LA BRASSINNE ALFRED DETHIER CLAUDINE COUTEAUXDUMONT.
CRYSTAL-LIKE INCLUSIONS IN HEART MITOCHONDRIA
SIR,-Intramitochondrial paracrystalloid inclusions have been described in several organs in various liver diseases,l-3 disturbances of lipid metabolism,2, myopathy,5,6and primary heart hypertrophy,7 and in epithelial cells of the renal tubules after vasopressin administrationand mitochondria of intact bovine heart.9 Opinions differ about the
::.-
i
Fig. 2-Inclusions in isolated heart mitochondria (x71,300, insert x 108,000).
We have studied the effect of several specific respiratory inhibitors and have found that mitochondrial membranes are not altered after treatment with 10-4M potassium cyanide, 10-4M malonat, or 4 x 10-6M oligomycin. Nevertheless, we have observed crystal-like substances in isolated and non-isolated heart mitochondria incubated in Locke solution containing 4 x 10-4M 2,4-dinitrophenol (D.N.P.) at 37°C for 45 minutes. In 10-15% of the mitochondria cristm were elongated. Between the inner membranes an electron-dense substance partly of fine periodicity was found (fig. 1). Longitudinal sections revealed two membranes about 20 A wide lying about 60 A apart (fig. 2). The inclusions also developed in a calcium-free medium containing 10-3 M edetic acid. We think that these bodies are evidence of non-specific damage to the mitochondrial metabolism. We assume that in their development disorganisation of the oxidative chain may play a part. The effect of essential calcium accumulation may be excluded. Further investigations are needed to reveal the nature of the substance and the exact site of the biochemical disturbance.
Fig. 1-Crystalline inclusions in mitochondria of myocardial pieces (reduced by three-quarters from x 18,630).
and structure of the substance. Disturbances of lipid metabolism might play a part.2,4 Damage to the mitochondrial oxidative system has been suggested by GomezLazaro and his colleagues (Dec. 30, p. 1417) and others.8,9 The latter suggestion is compatible with their protein nature and enzyme activity.2 The possibility of calcium accumulation, at least in heart mitochondria, has been suggested. We have found similar inclusions in pacemaker-stimulated rabbit heart. The cardiac mitochondria isolated from the area of the lesion showed evidence of disordered respiration. 1o
origin
Wills, E. J. J. Cell Biol. 1965, 24, 511. Bhagwat, A. G., Ross, R. C. Archs Path. 1971, 91, 70. Gomez-Lázaro, G., Caro-Paton, A., Prieto, J., Coca, M. C. Lancet, 1972, ii, 1471. 4. Kovacs, K., Lee, R., Little, J. A. ibid. 1972, i, 752. 5. Salmon, M. A., Esiri, M. M., Ruderman, N. B. ibid. 1971, ii, 290. 6. Bradley, W. G., Hudgson, P., Gardner-Medwin, D., Walton, J. N. ibid. 1969, i, 495. 7. Soos, J., Sótonyi, P., Szinay, Gy. Morph. Ig. Orv. Szle, 1972, 12,
1. 2. 3.
263. 8. Sun, C. N., White, H. J., Towbin, E. J. Exp. Path. 1972, 7, 166. 9. Hall, J. D., Crane, F. E. J. Cell Biol. 1971, 48, 420. 10. Somogyi, E., Sotonyi, P., Bujdosó, Gy., Nemes, A. Z. Rechtsmed. 1971, 68, 180.
p ,N, . ,..
Department of Forensic Medicine, Semmelweis University of Medicine, 1450 Budapest Pf. 9/41, Hungary.
E. SOMOGYI T. VARGA G. BUJDOSÓ P. SOTONYI.
EYE AND SKIN LESIONS IN TYROSINÆMIA
SIR,-Keratitis and dermatological lesions of the hands are not seen in infants with neonatal hypertyrosinaemia or in patients with the usual type of hereditary tyrosinaemia (type-I) which is associated with cirrhosis and renal damage.! We have lately reported a patient with a and feet
tyrosinæmia, in whom the enzyme deficiency of cytoplasmic tyrosineaminotransferase activity.2,3 This patient differed from other patients with hereditary tyrosinæmia in a number of ways, including the fact that liver and kidney functions were not impaired. Our patient exhibited a number of different form of
abnormality
was
unusual clinical 1. 2. 3.
a
findings,
among which
were severe
and
Fellman, J. H., Vanbellinghen, P. J., Jones, R. T., Koler, R. D. Biochemistry, N.Y. 1969, 8, 615. Kennaway, N. G., Buist, N. R. M. Pediat. Res. 1971, 5, 287. LaDu, B. N., Gjessing, L. R. in Metabolic Basis of Inherited Disease (edited by J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson); p. 296. New York, 1972.
DEVOITILLE.
University of Liège, Belgium
JEAN-MICHEL
Department of Dermatology, Hôpital de Bavière, University of Liège, Belgium
MICHEL-F. DE LA BRASSINNE ALFRED DETHIER CLAUDINE COUTEAUXDUMONT.
CRYSTAL-LIKE INCLUSIONS IN HEART MITOCHONDRIA
SIR,-Intramitochondrial paracrystalloid inclusions have been described in several organs in various liver diseases,l-3 disturbances of lipid metabolism,2, myopathy,5,6and primary heart hypertrophy,7 and in epithelial cells of the renal tubules after vasopressin administrationand mitochondria of intact bovine heart.9 Opinions differ about the
::.-
i
Fig. 2-Inclusions in isolated heart mitochondria (x71,300, insert x 108,000).
We have studied the effect of several specific respiratory inhibitors and have found that mitochondrial membranes are not altered after treatment with 10-4M potassium cyanide, 10-4M malonat, or 4 x 10-6M oligomycin. Nevertheless, we have observed crystal-like substances in isolated and non-isolated heart mitochondria incubated in Locke solution containing 4 x 10-4M 2,4-dinitrophenol (D.N.P.) at 37°C for 45 minutes. In 10-15% of the mitochondria cristm were elongated. Between the inner membranes an electron-dense substance partly of fine periodicity was found (fig. 1). Longitudinal sections revealed two membranes about 20 A wide lying about 60 A apart (fig. 2). The inclusions also developed in a calcium-free medium containing 10-3 M edetic acid. We think that these bodies are evidence of non-specific damage to the mitochondrial metabolism. We assume that in their development disorganisation of the oxidative chain may play a part. The effect of essential calcium accumulation may be excluded. Further investigations are needed to reveal the nature of the substance and the exact site of the biochemical disturbance.
Fig. 1-Crystalline inclusions in mitochondria of myocardial pieces (reduced by three-quarters from x 18,630).
and structure of the substance. Disturbances of lipid metabolism might play a part.2,4 Damage to the mitochondrial oxidative system has been suggested by GomezLazaro and his colleagues (Dec. 30, p. 1417) and others.8,9 The latter suggestion is compatible with their protein nature and enzyme activity.2 The possibility of calcium accumulation, at least in heart mitochondria, has been suggested. We have found similar inclusions in pacemaker-stimulated rabbit heart. The cardiac mitochondria isolated from the area of the lesion showed evidence of disordered respiration. 1o
origin
Wills, E. J. J. Cell Biol. 1965, 24, 511. Bhagwat, A. G., Ross, R. C. Archs Path. 1971, 91, 70. Gomez-Lázaro, G., Caro-Paton, A., Prieto, J., Coca, M. C. Lancet, 1972, ii, 1471. 4. Kovacs, K., Lee, R., Little, J. A. ibid. 1972, i, 752. 5. Salmon, M. A., Esiri, M. M., Ruderman, N. B. ibid. 1971, ii, 290. 6. Bradley, W. G., Hudgson, P., Gardner-Medwin, D., Walton, J. N. ibid. 1969, i, 495. 7. Soos, J., Sótonyi, P., Szinay, Gy. Morph. Ig. Orv. Szle, 1972, 12,
1. 2. 3.
263. 8. Sun, C. N., White, H. J., Towbin, E. J. Exp. Path. 1972, 7, 166. 9. Hall, J. D., Crane, F. E. J. Cell Biol. 1971, 48, 420. 10. Somogyi, E., Sotonyi, P., Bujdosó, Gy., Nemes, A. Z. Rechtsmed. 1971, 68, 180.
p ,N, . ,..
Department of Forensic Medicine, Semmelweis University of Medicine, 1450 Budapest Pf. 9/41, Hungary.
E. SOMOGYI T. VARGA G. BUJDOSÓ P. SOTONYI.
EYE AND SKIN LESIONS IN TYROSINÆMIA
SIR,-Keratitis and dermatological lesions of the hands are not seen in infants with neonatal hypertyrosinaemia or in patients with the usual type of hereditary tyrosinaemia (type-I) which is associated with cirrhosis and renal damage.! We have lately reported a patient with a and feet
tyrosinæmia, in whom the enzyme deficiency of cytoplasmic tyrosineaminotransferase activity.2,3 This patient differed from other patients with hereditary tyrosinæmia in a number of ways, including the fact that liver and kidney functions were not impaired. Our patient exhibited a number of different form of
abnormality
was
unusual clinical 1. 2. 3.
a
findings,
among which
were severe
and
Fellman, J. H., Vanbellinghen, P. J., Jones, R. T., Koler, R. D. Biochemistry, N.Y. 1969, 8, 615. Kennaway, N. G., Buist, N. R. M. Pediat. Res. 1971, 5, 287. LaDu, B. N., Gjessing, L. R. in Metabolic Basis of Inherited Disease (edited by J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson); p. 296. New York, 1972.