firmed the current concept of pathogenesis of LAM: TSC-LAM has a germline mutation but sporadic LAM does not; sporadic LAM is a TSC2 disease with two somatic mutations; and a variety of TSC mutations causes LAM. However, our study indicates that a fraction of sporadic LAM can be a TSC1 disease; therefore, both TSC genes should be examined, even for patients with sporadic LAM.

Small dorsal root ganglion neurons express preferentially the Na+ channel isoform Na(v)1.9 that mediates a tetrodotoxin-resistant (TTX-R) Na+ current. We investigated properties of the Na+ current mediated by Na(v)1.9 (I(NaN)) using the whole-cell, patch-clamp recording technique. To isolate I(NaN) from heterogeneous TTX-R Na+ currents that also contain another type of TTX-R Na+ current mediated by Na(v)1.8, we used Na(v)1.8-null mutant mice. When F- was used as an internal anion in the patch pipette solution, both the activation and inactivation kinetics for I(NaN) shifted in the hyperpolarizing direction with time. Such a time-dependent shift of the kinetics was not observed when Cl- was used as an internal anion. Functional expression of I(NaN) declined with time after cell dissociation and recovered during culture, implying that Na(v)1.9 may be regulated dynamically by trophic factors or depend on subtle environmental factors for its survival. During whole-cell recordings, the peak amplitude of I(NaN) increased dramatically after a variable delay, as if inactive or silent channels had been "kindled". Such an unusual increase of the amplitude could be prevented by adding ATP to the pipette solution or by recording with the nystatin-perforated patch-clamp technique, suggesting that the rupture of patch membrane affected the behaviour of Na(v)1.9. These peculiar properties of I(NaN) may provide an insight into the plasticity of Na+ channels that are related to pathological functions of Na+ channels accompanying abnormal pain states.

These data support bypass graft surgery in patients with severe LV dysfunction. With careful cardioplegic techniques, hospital mortality was low (3.8%). Long-term survival is encouraging, with good relief of symptoms in most patients. Perioperative VAs are frequent but respond to medical treatment, with only 23% of patients discharged on antiarrhythmic drugs. Five-year freedom from sudden death is 91%, with only 3 late sudden deaths in this series.

A diamond X-ray phase retarder has been combined with a phase-sensitive detection method to improve the accuracy of X-ray magnetic circular dichroism (XMCD) measurements. Fluctuation of the absorption coefficient of a magnetized sample was induced by photon-helicity switching at 40 Hz by flipping the phase retarder, and directly detected through a lock-in amplifier referring to the helicity-modulation frequency. This new technique for XMCD measurements was applied to the Fe K-edge in pure Fe foil. XMCD spectra of higher signal-to-noise ratio were obtained in shorter measuring time.

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