Case summary
When skin biopsies were taken from our patient with lipoatrophic diabetes the young female was 22 years of age. She had a body weight of 30 kg, the body length was 163 cm. The first signs of lipodystrophy were seen in the third year of her life. About 4 years later the loss of subcutaneous fat was complete. The patient suffered from acanthosis nigricans when she was 11 years of age. At that time her case was described especially for its dermatological aspects (3). When at the age of 15 years the patient was admitted to our hospital diabetes mellitus with blood sugar levels about 400 mg/dl and excessive glucosuria had developed. Insulin did not influence the blood sugar levels but lowered the plasma levels of the ketone bodies (5). We could show a nearly normal response of the endogenous insulin production to an hypoglycemic stimulus. All known endocrine disorders which influence the action of insulin could be excluded. There were no elevated levels of insulin antibodies.
Control cultures of fibroblast were obtained from an 11 month old Puerto Rican girl-Institute for Medical Research, Camden, NY, Code GM 311-(Co 1), from a 14 years old white girl (Co 2), from a 24 years (Co 3) and 29 years old white man (Co 4). They all were of normal body weight and had no detectable metabolid disorder.
Similar content being viewed by others
References
ARCHER, J.A., GORDON, P., GAVIN III, J.R., LESNIAK, M.A., ROTH, J.: Insulin receptors in human circulating lymphocytes: Application to the study of insulin resistance in man. J. Clin. Endocrinol. Metab.36, 627–633 (1973)
ARCHER, J.A., GORDON, P., ROTH, J.: Defect in insulin binding to receptors in obese man. J. Clin. Invest.55, 166–174 (1975)
BANDMANN, H.-J., ROMITI, N., STEHR, K.: Beitrag zur Klinik und zur Klassifizierung der Acanthosis nigricans. Hautarzt16, 492–498 (1965)
BRUBAKER, M.M., LEVAN, N.E., COLLIP, P.J.: Acanthosis nigricans and congenital total lipodystrophy. Arch. Derm.91, 320–325 (1965)
DÖRFLER, H., WOLFRAM, G., HEPP, K.D.: Ketogenese beim lipatrophischen Diabetes and ihre Beeinflussung durch Insulin. Verh. Dtsch. Ges. inn. Med.82, 782–785 (1976)
GAVIN III, J.R., GORDON, P., ROTH, J., ARCHER, J.A., BUELL, D.N.: Characteristics of the human lymphocyte insulin receptor. J. Biol. Chem.248, 2202–2207 (1973)
GREEN, H., KEHINDE, O.: An established preadipose cell line and its differentiation in culture. Cell5, 19–27 (1975)
GUIHARD, J., TESSIER, R., LANIECE, M., FOUCAULT, J.-P., SIBIREFF, J.-J., L'HIRONDEL, J.: Diabète lipoatrophique (syndrome de Lawrence). Ann. Pediat.18, 633–643 (1971)
HOLLENBERG, M.D., CUATRECASAS, P.: Insulin and epidermal growth factor. J. Biol. Chem.250, 3845–3853 (1974)
HSIA, D.Y.-Y.: Study of hereditary metabolic disease using in vitro techniques. Metabolism19, 309–339 (1970)
KAHN, C.R., FLIER, J.S., BAR, R.S., ARCHER, J.A., GORDON, P., MARTIN, M.M., ROTH, J.: The syndromes of insulin resistance and acanthosis nigricans- insulin - receptor disorders in man. N. Engl. J. Med.294, 739–745 (1976)
LAWRENCE, R.D.: Lipodystrophy and hepatomegaly with diabetes, lipemia and other metabolic disturbances. Lancet 1946/I, 724–731; 773–775
RECHLER, M.M., PODSKALNY, J.M.: Insulin receptors in cultured human fibroblasts. Diabetes25, 250–255 (1976)
REED, W.B., DEXTER, R., CORLEY, CH., FISH, CH.: Congenital lipoatrophic diabetes with acanthosis nigricans. Arch. Derm. Syph. (Chic.)91, 326–332 (1965)
STEIN, O., STEIN, Y., WEINSTEIN, D., STEINBERG, D.: Binding, internalisation and degradation of low density lipoproteins by normal fibroblasts and by fibroblasts of a case of homocygotic familial hypercholesterolemia. Proc. Natl. Acad. Sci. USA73, 14–18 (1974)
ZÖLLNER, N., DÖRFLER, H., WOLFRAM, G.: Lipatrophischer Diabetes - ein Fallbericht. Münch. med. Wschr.117, 1209–1214 (1975)
Author information
Authors and Affiliations
Additional information
This work has been supported by the Deutsche Forschungsgemeinschaft
Lipoatrophic diabetes - synonymous generalized lipodystrophy - is a rare syndrome but an important model for the understanding of some common metabolic diseases, e.g. diabetes mellitus. The disease is characterized by: Complete lack of subcutaneous adipose tissue, insulin resistant diabetes mellitus, hepatosplenomegaly, excessive hyperlipidemia, elevated basal metabolic rate without hyperthyroidism.
We studied the binding of insulin to cultured fibroblasts of a patient, who showed all signs of the disease (16).
Rights and permissions
About this article
Cite this article
Dörfler, H., Wieczorek, A., Wolfram, G. et al. Binding of insulin to fibroblasts in lipoatrophic diabetes. Res. Exp. Med. 170, 161–168 (1977). https://doi.org/10.1007/BF01851387
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF01851387