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The clinical and physiological aspects of tuftsin deficiency syndromes exhibiting defective phagocytosis

Klinische und physiologische aspekte von Tuftsin-Mangelsyndromen mit gestörter Phagocytose

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Zusammenfassung

Das Tetrapeptid Tuftsin (Thr-Lys-Pro-Arg) stimuliert gezielt die Phagozytose von neutrophilen Granulozyten und Gewebsmakrophagen. Tuftsin in seiner freien aktiven Form wird durch zwei Enzyme vom Träger-γ-globulin abgespalten.

Kongenitale Tuftsindefizienz kommt durch Mutationen zustande, deren Ergebnis ein inaktives Peptid ist. Die erworbene Tuftsindefizienz wird beobachtet, wenn die Funktionen der Milz durch die Entfernung des Organs oder durch Krankheit eingeschränkt sind. Eine Tuftsindefizienz macht sich durch schwere rekurrierende Infektionen besonders der Haut, der Lymphknoten und der Lungen bemerkbar. Die Therapie beschränkt sich auf γ-Globulininjektionen und entsprechende Antibiotikagaben.

Summary

The tetrapeptide tuftsin (Thr-Lys-Pro-Arg) stimulates phagocytosis by blood neutrophilic granulocytes and tissue macrophages in a highly specific manner. Tuftsin is cleaved off the carrier γ-globulin molecule as the free active form by two enzymes. One of these is in the spleen and the other on the outer membrane of the phagocyte.

Congenital tuftsin deficiency usually arises when the peptide is mutated to an inactive peptide. The acquired type occurs if the spleen function is curtailed by removal or disease. Tuftsin deficiency is manifested by severe recurrent infections involving primarily the skin, lymph nodes and lungs. Therapy is limited to γ-globulin injection along with appropriate chemotherapy.

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  1. Division of Protein Chemistry, Tufts University School of Medicine, Boston

    V. A. Najjar

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Najjar, V.A. The clinical and physiological aspects of tuftsin deficiency syndromes exhibiting defective phagocytosis. Klin Wochenschr 57, 751–756 (1979). https://doi.org/10.1007/BF01478032

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