Effects of testosterone and methallibure on courtship and plasma androgen concentrations of male newts, Taricha granulosa

The administration of methallibure to male rough-skinned newts, Taricha granulosa, reduced the incidence of male courtship behavior and reduced concentrations of plasma androgens, as measured by radioimmunoassay. Daily injections of testosterone raised the concentration of plasma androgens above that in saline-treated males; however, testosterone treatments failed to moderate the inhibitory effect of methallibure on the expression of male mating behavior. The results indicate that male sexual responsiveness in Taricha is influenced by changes in hormones other than plasma androgens.     

Distinct variants of erythrocyte protein 4.1 inherited in linkage with elliptocytosis and Rh type in three white families

Hereditary elliptocytosis is a heterogeneous disorder resulting from defects in the erythrocyte membrane skeleton. Although some cases of elliptocytosis result from defects in spectrin, the specific structural abnormality has yet to be identified in the majority of cases. Protein 4.1 plays an essential role in erythrocyte membrane physiology, and deficiencies have been implicated in only a few rare cases of elliptocytosis. By using 4.1 immunoblots and a 4.1 radioimmunoassay we identified distinct variants of protein 4.1 in 15 elliptocytic members of three US white families with the Rh-linked form of elliptocytosis. Elliptocytic members of family G were heterozygotes for a low-molecular weight (mol wt) 4.1 variant (65,000 to 68,000 daltons; normal, 80,000) inherited in linkage with the Rz phenotype. Elliptocytic members of family C expressed a simple partial deficiency of protein 4.1 (63% of the normal level) that was inherited in linkage with the r phenotype. Elliptocytic members of family N were heterozygotes for a high-mol wt 4.1 variant (100,000 daltons) also inherited in linkage with the r phenotype. These studies indicate that mutant forms of protein 4.1 are not uncommon in elliptocytosis among whites and that different kindreds probably express different mutations. The observed linkage of elliptocytosis and Rh blood type most likely results from the close proximities of the 4.1 gene (site of the mutation) and the Rh gene, which is located nearby on the short arm of chromosome 1.     

HLA antigens in ankylosing spondylitis associated with Crohn's disease. Increased frequency of the HLA phenotype B27,B44

In a consecutive case study 231 patients with Crohn's disease were investigated for ankylosing spondylitis (AS) and HLA-A, B, C, DR antigen association. Eighteen patients (7.8%) had definite AS according to the New York criteria; 13 (72%) were HLA-B27 positive. The phenotype B27,B44 was seen in 8 patients (44%) compared to only 3 (1%) of 300 controls (p less than 10(-7), and 1 (0.5%) of 213 patients with Crohn's disease without AS (p less than 10(-7). We conclude that patients with the phenotype B27,B44 are highly at risk (relative risk = 68.8) for the common manifestation of Crohn's disease and AS.     

Metamorphosis in the summer flounder, Paralichthys dentatus: thyroidal status influences gill mitochondria-rich cells

Metamorphosis in the summer flounder (Paralichthys dentatus) is mediated by thyroid hormones (TH) and is accompanied by changes in gill mitochondria-rich cells (MRCs) and in salinity tolerance. Altered thyroid status during larval development and metamorphosis in this species influences salinity tolerance, though the influence of any hormone on MRCs of larval marine teleosts is not known. This study characterized the effect of altered thyroid status on MRC intracellular membranes, mitochondria size and ultrastructure, immunoreactive (ir)-Na(+),K(+)-ATPase, and cell size and density during metamorphosis in summer flounder. Inhibition of metamorphosis with thiourea (30 ppm) (TU, an inhibitor of TH synthesis) inhibited changes in MRCs, producing large "larval" type MRCs with weak reactivity to osmium; large, electron-lucent mitochondria; and weak ir-Na(+),K(+)-ATPase. Replacement of TH with TU + thyroxine-Na salt (100 ppb) rescued the fish from developmental inhibition, producing smaller "juvenile" type MRCs with strong reactivity to osmium; smaller, electron-opaque mitochondria; and strong ir-Na(+), K(+)-ATPase. The findings suggest that TH are necessary for MRCs to change from larval to juvenile form during metamorphosis.     

Erythroid failure in Diamond-Blackfan anemia is characterized by apoptosis

Programmed cell death, also known as apoptosis, is frequently initiated when cells are deprived of specific trophic factors. To investigate if accelerated apoptosis contributes to the pathogenesis of Diamond- Blackfan anemia (DBA), a rare pure red blood cell aplasia of childhood, we studied the effect of erythropoietin (epo) deprivation on erythroid progenitors and precursors from the bone marrow of DBA patients as compared with hematologically normal controls. Apoptosis in response to epo deprivation was evaluated by enumeration of colony-forming unit- erythroid (CFU-E)- and burst-forming unit-erythroid (BFU-E)-derived colonies in plasma clot semisolid culture and by the identification of typical DNA oligosomes by gel electrophoresis from marrow mononuclear cells in liquid culture. In all DBA patients there was a marked decrease in CFU-E- and BFU-E-derived colony formation compared with normal controls at comparable time points of epo deprivation, with a complete loss of CFU-E-derived colonies in semisolid culture by 9 hours of epo deprivation versus 48 hours in controls. The BFU-E-derived colony response to epo deprivation displayed a similar pattern of decrement. Apoptotic changes assessed by the presence of characteristic DNA fragmentation began in the absence of epo deprivation and were readily detected within 3 hours of epo deprivation in DBA cultures versus 9 hours in controls. We conclude that DBA is characterized by accelerated apoptosis as measured by the loss of erythroid progenitor clonogenicity and increased progenitor and precursor DNA fragmentation leading to the formation of characteristic oligosomes, consistent with an intrinsic erythroid-progenitor defect in which increased sensitivity to epo deprivation results in erythroid failure.