14 research outputs found

    Dictyostelium LvsB Mutants Model the Lysosomal Defects Associated with Chediak-Higashi Syndrome

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    Chediak-Higashi syndrome is a genetic disorder caused by mutations in a gene encoding a protein named LYST in humans (“lysosomal trafficking regulator”) or Beige in mice. A prominent feature of this disease is the accumulation of enlarged lysosome-related granules in a variety of cells. The genome of Dictyostelium discoideum contains six genes encoding proteins that are related to LYST/Beige in amino acid sequence, and disruption of one of these genes, lvsA (large volume sphere), results in profound defects in cytokinesis. To better understand the function of this family of proteins in membrane trafficking, we have analyzed mutants disrupted in lvsA, lvsB, lvsC, lvsD, lvsE, and lvsF. Of all these, only lvsA and lvsB mutants displayed interesting phenotypes in our assays. lvsA-null cells exhibited defects in phagocytosis and contained abnormal looking contractile vacuole membranes. Loss of LvsB, the Dictyostelium protein most similar to LYST/Beige, resulted in the formation of enlarged vesicles that by multiple criteria appeared to be acidic lysosomes. The rates of endocytosis, phagocytosis, and fluid phase exocytosis were normal in lvsB-null cells. Also, the rates of processing and the efficiency of targeting of lysosomal α-mannosidase were normal, although lvsB mutants inefficiently retained α-mannosidase, as well as two other lysosomal cysteine proteinases. Finally, results of pulse-chase experiments indicated that an increase in fusion rates accounted for the enlarged lysosomes in lvsB-null cells, suggesting that LvsB acts as a negative regulator of fusion. Our results support the notion that LvsB/LYST/Beige function in a similar manner to regulate lysosome biogenesis

    Asteroseismology of RXJ 2117+3412, the hottest pulsating PG 1159 star

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    The pulsating PG 1159 planetary nebula central star RXJ 2117+3412 has been observed over three successive seasons of a multisite photometric campaign. The asteroseismological analysis of the data, based on the 37 identified l = 1 modes among the 48 independent pulsation frequencies detected in the power spectrum, leads to the derivation of the rotational splitting, the period spacing and the mode trapping cycle and amplitude, from which a number of fundamental parameters can be deduced. The average rotation period is 1.16 ± 0.05 days. The trend for the rotational splitting to decrease with increasing periods is incompatible with a solid body rotation. The total mass is 0.56⁺⁰˙⁰²₋₀.₀₄ Mѳ and the He-rich envelope mass fraction is in the range 0.013–0.078 M*. The luminosity derived from asteroseismology is log(L/Lѳ) = 4.05⁺⁰˙²³₋₀.₃₂ and the distance 760⁺²³⁰₋₂₃₅ pc. At such a distance, the linear size of the planetary nebulae is 2.9 ± 0.9 pc. The role of mass loss on the excitation mechanism and its consequence on the amplitude variations is discussed

    Asteroseismology of RXJ 2117+3412, the hottest pulsating PG 1159 star

    Get PDF
    The pulsating PG 1159 planetary nebula central star RXJ 2117+3412 has been observed over three successive seasons of a multisite photometric campaign. The asteroseismological analysis of the data, based on the 37 identified l = 1 modes among the 48 independent pulsation frequencies detected in the power spectrum, leads to the derivation of the rotational splitting, the period spacing and the mode trapping cycle and amplitude, from which a number of fundamental parameters can be deduced. The average rotation period is 1.16 ± 0.05 days. The trend for the rotational splitting to decrease with increasing periods is incompatible with a solid body rotation. The total mass is 0.56⁺⁰˙⁰²₋₀.₀₄ Mѳ and the He-rich envelope mass fraction is in the range 0.013–0.078 M*. The luminosity derived from asteroseismology is log(L/Lѳ) = 4.05⁺⁰˙²³₋₀.₃₂ and the distance 760⁺²³⁰₋₂₃₅ pc. At such a distance, the linear size of the planetary nebulae is 2.9 ± 0.9 pc. The role of mass loss on the excitation mechanism and its consequence on the amplitude variations is discussed
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