X-ALD iPSC model recapitulates the key events of disease development (ie, VLCFA accumulation in oligodendrocytes), provides new clues for better understanding of the disease, and allows for early and accurate diagnosis of the disease subtypes. X-ALD oligodendrocytes can be a useful cell model system to develop new therapeutics for treating X-ALD.

Facial EMG monitoring of the LSR is an effective tool to use when performing complete decompression, and it may be helpful in predicting outcomes.

Our findings indicate that yoga exercise improves adiponectin level, serum lipids, and metabolic syndrome risk factors in obese postmenopausal women. Consequently, yoga exercise will be effective in preventing cardiovascular disease caused by obesity in obese postmenopausal Korean women.

The generation of disease-specific induced pluripotent stem cell (iPSC) lines from patients with incurable diseases is a promising approach for studying disease mechanisms and drug screening. Such innovation enables to obtain autologous cell sources in regenerative medicine. Herein, we report the generation and characterization of iPSCs from fibroblasts of patients with sporadic or familial diseases, including Parkinson's disease (PD), Alzheimer's disease (AD), juvenile-onset, type I diabetes mellitus (JDM), and Duchenne type muscular dystrophy (DMD), as well as from normal human fibroblasts (WT). As an example to modeling disease using disease-specific iPSCs, we also discuss the previously established childhood cerebral adrenoleukodystrophy (CCALD)- and adrenomyeloneuropathy (AMN)-iPSCs by our group. Through DNA fingerprinting analysis, the origins of generated disease-specific iPSC lines were identified. Each iPSC line exhibited an intense alkaline phosphatase activity, expression of pluripotent markers, and the potential to differentiate into all three embryonic germ layers: the ectoderm, endoderm, and mesoderm. Expression of endogenous pluripotent markers and downregulation of retrovirus-delivered transgenes [OCT4 (POU5F1), SOX2, KLF4, and c-MYC] were observed in the generated iPSCs. Collectively, our results demonstrated that disease-specific iPSC lines characteristically resembled hESC lines. Furthermore, we were able to differentiate PD-iPSCs, one of the disease-specific-iPSC lines we generated, into dopaminergic (DA) neurons, the cell type mostly affected by PD. These PD-specific DA neurons along with other examples of cell models derived from disease-specific iPSCs would provide a powerful platform for examining the pathophysiology of relevant diseases at the cellular and molecular levels and for developing new drugs and therapeutic regimens.

The disappearance of the LSR before and after MVD may predict clinical outcomes. This must be considered together with the disappearance or persistence of LSR after decompression as a prognostic factor for HFS after MVD.

Microvascular decompression (MVD) is an effective and safe treatment option that offers the prospect of definitive cure for hemifacial spasm (HFS). However, there are potential risks of complications for MVD associated with retromastoid suboccipital craniectomy (RmSOC) and cranial nerves in particular. The purpose of this study was to identify clinical characteristics of possible complications after MVD for HFS and to establish appropriate management concept of these complications. We retrospectively reviewed medical records of 2040 patients who underwent RmSOC with MVD for HFS at Samsung medical center between January 1998 and March 2013. Of 2040 patients, 2027 were followed (99.4%). Of the 2027 patients, 1841 (90.8%) exhibited complete relief or minimal symptoms, and 113 (5.6%) reported improved spasm but had mild remnant symptoms. After operation, the most frequently developed complications were facial nerve palsy (8.19%), followed by middle ear effusion (4.90%) and hearing loss (3.63%). There were two cases of supratentorial subdural hemorrhage, three cases of infarction. MVD was found to be safe and effective treatment for HFS, in consistent with previous reports. Some of the complications such as facial nerve palsy, middle ear effusion, and hearing loss are relatively common. However, they have mild clinical courses that are usually transient.

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There is a crucial need for simple and highly sensitive techniques to detect DNA in complicated biological samples such as serum. Here we present an ultrasensitive electrochemical DNA sensor using (i) single DNA hybridization with peptide nucleic acid (PNA), (ii) selective binding of [Ru(NH(3))(6)](3+) to hybridized DNA, (iii) fast NaBH(4) electrooxidation mediated by [Ru(NH(3))(6)](3+), and (iv) low background currents of NaBH(4) at indium-tin oxide (ITO) electrodes. The [Ru(III)(NH(3))(5)NH(2)](2+) formed from [Ru(III)(NH(3))(6)](3+) in borate buffer (pH 11.0) is readily electrooxidized to both [Ru(IV)(NH(3))(5)NH(2)](3+) and Ru complex with a higher oxidation state. In the absence of [Ru(NH(3))(6)](3+) bound to the DNA-sensing ITO electrodes, the oxidation currents of NaBH(4) are very low. However, in the presence of [Ru(NH(3))(6)](3+), the oxidation currents of NaBH(4) are highly enhanced due to electron mediation of the oxidized Ru complexes. The significant enhancement in the electrocatalytic activity of sensing electrodes after [Ru(NH(3))(6)](3+) binding facilitates to obtain high signal-to-background ratios. PNA and ethylenediamine on DNA-sensing electrodes significantly decrease [Ru(NH(3))(6)](3+) binding, also allowing for high signal-to-background ratios. The oxidation charges of NaBH(4) obtained from chronocoulometry are highly reproducible. All combined effects enable the detection of DNA with a detection limit of 1 fM in ten-fold diluted human serum. The simple and fast detection procedure and the ultrasensitivity make this approach highly promising for practical DNA detection.