Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome.

Purpose: This study evaluated the safety and effectiveness of long-term enzyme replacement therapy with idursulfase (recombinant human iduronate-2-sulfatase) in patients with Hunter syndrome.

Methods: All 94 patients who completed a 53-week double-blinded study of idursulfase enrolled in this open-labeled extension study and received intravenous idursulfase at a dose of 0.5 mg/kg weekly for 2 years, and clinical outcomes and safety were assessed.

Happiness unpacked: positive emotions increase life satisfaction by building resilience.

Happiness-a composite of life satisfaction, coping resources, and positive emotions-predicts desirable life outcomes in many domains. The broaden-and-build theory suggests that this is because positive emotions help people build lasting resources. To test this hypothesis, the authors measured emotions daily for 1 month in a sample of students (N = 86) and assessed life satisfaction and trait resilience at the beginning and end of the month.

Emotional resilience in early childhood: developmental antecedents and relations to behavior problems.

To test whether the development of emotional resilience is a function of sensitive caregiving and child negative affect, we tested the joint contributions of 7-month maternal sensitivity and infant negative affect to the prediction of 33-month emotional resilience across the first 3 years of life. The aims of this study were to examine whether maternal sensitivity and infant negative affect predict long-term emotional resilience and whether this was associated with preschool behavior problems. Using a sample of 181 mother-infant dyads, we found that (a) maternal sensitivity at 7 months, but not infant negative affect, longitudinally predicted emotional resilience during preschool and (b) emotional resilience was negatively associated with anxiety/depression in preschool.

A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome).

Purpose: To evaluate the safety and efficacy of recombinant human iduronate-2-sulfatase (idursulfase) in the treatment of mucopolysaccharidosis II.

Methods: Ninety-six mucopolysaccharidosis II patients between 5 and 31 years of age were enrolled in a double-blind, placebo-controlled trial. Patients were randomized to placebo infusions, weekly idursulfase (0.

Crosstalk between Rab GTPases and cell junctions.

For the past several years, studies from other laboratories, as well as ours, have begun to unravel the mechanism of germ cell movement in the testis by using several in vitro and in vivo models of tight and adherens junction assembly and disassembly, two cellular phenomena that confer cell movement. However, for cell movement to be fully appreciated, the importance of "intracellular" cell movements, such as those involving actin and microtubule filaments, must be better understood. Recent research on Rab GTPases has shown that members of this superfamily function in the trafficking of vesicles containing cargo to distinct subcellular sites such as the plasma membrane while utilizing actin and microtubule filaments as tracks.

Girls, aggression, and emotion regulation.

Many studies have demonstrated that boys are more aggressive than girls (see J. D. Coie & K.

Zyxin, axin, and Wiskott-Aldrich syndrome protein are adaptors that link the cadherin/catenin protein complex to the cytoskeleton at adherens junctions in the seminiferous epithelium of the rat testis.

During spermatogenesis, the movement of germ cells across the seminiferous epithelium is associated with extensive junction restructuring. Yet the underlying mechanism (or mechanisms) that regulates these events is largely unknown. If the molecular architecture of the cell-cell actin-based adherens junction (AJ), such as ectoplasmic specialization (ES) and tubulobulbar complex- two testis-specific AJ types, is known, many functional mechanistic studies can be designed.

Role of tissue inhibitor of metalloproteases-1 in junction dynamics in the testis.

Using multiple high-performance liquid chromatography steps, we have identified and purified a polypeptide to apparent homogeneity from primary Sertoli cell conditioned culture medium that consisted of 2 molecular variants of 31 and 29 kDa when electrophoresed on a sodium dodecyl sulfate-polyacrylamide gel run under reducing conditions. Partial N-terminal amino acid sequence analysis of these 2 proteins revealed a sequence of NH(2)-IKMAKMLKGFDAVGNATG, which is homologous to tissue inhibitor of metalloproteases-1 (TIMP-1). Studies by semiquantitative reverse transcription-polymerase chain reaction using a primer pair specific to rat TIMP-1 demonstrated that both Sertoli and germ cells express TIMP-1.