Inherited disorders in sexual development

Ascariasis is a well-known cause of acute cholangitis and cholecystitis; however, very rarely do worms penetrate and colonize the liver parenchyma. Here we present the unique radiological demonstration (US, CT and ERCP) of hepatobiliary ascariasis in which worm was first alive in the parenchyma of the liver and subsequently died and formed liver abscess.     

Granulocytic sarcoma presented as a reactivation of chronic myeloid leukemia after allogenic marrow transplantation

The authors report the case of a chronic myeloid leukemia (CML) patient submitted to allogenic bone marrow transplantation, who had probably never entered complete remission. The disease was reactivated as a granulocytic sarcoma, next to a platinum plate installed to correct a tibia fracture 11 years earlier. Its final event was a myeloid Ph1 + blastic crisis that was unsuccessfully treated with high doses of sc interferon and citarabine.     

ATP-dependent proteolysis in mitochondria. m-AAA protease and PIM1 protease exert overlapping substrate specificities and cooperate with the mtHsp70 system

To analyze protein degradation in mitochondria and the role of molecular chaperone proteins in this process, bovine apocytochrome P450scc was employed as a model protein. When imported into isolated yeast mitochondria, P450scc was mislocalized to the matrix and rapidly degraded. This proteolytic breakdown was mediated by the ATP-dependent PIM1 protease, a Lon-like protease in the mitochondrial matrix, in cooperation with the mtHsp70 system. In addition, a derivative of P450scc was studied to which a heterologous transmembrane region was fused at the amino terminus. This protein became anchored to the inner membrane upon import and was degraded by the membrane-embedded, ATP-dependent m-AAA protease. Again, degradation depended on the mtHsp70 system; it was inhibited at non-permissive temperature in mitochondria carrying temperature-sensitive mutant forms of Ssc1p, Mdj1p, or Mge1p. These results demonstrate overlapping substrate specificities of PIM1 and the m-AAA protease, and they assign a central role to the mtHsp70 system during the degradation of misfolded polypeptides by both proteases.     

Activity of threonine deaminase and biosynthesis of avermectins in the culture of Streptomyces avermitilis

The influence of ammonium, threonine, isoleucine and valine on the activity of threonine deaminase and the biosynthesis of avermectins in the culture of two mutants of Streptomyces avermitilis, i.e. a sensitive one and a resistant one with respect to alpha-amino-beta-oxyvaleric acid, a threonine antimetabolite, was studied. It was shown that the synthesis of threonine deaminase was induced by threonine and valine in the mycelium of both the mutants. The level of threonine deaminase was higher in the mycelium of the antimetabolite resistant mutant. The antibiotic activity of the resistant mutant was lower while the relative content of the group B avermectins in the pool of the synthesized avermectins was higher than that in the culture of the sensitive mutant.