Introduction: Membranous aplasia cutis congenita (MACC) is the most common clinical subtype of aplasia cutis congenita (ACC). It is typified by a localized skin lesion devoid of hair and features a membranous surface. While most MACC individuals do not present with concurrent abnormalities, it can sometimes co-occur with additional physical anomalies and various malformation syndromes.
View Article and Find Full Text PDFMolecular mechanisms underlying the repair of nitrosylated [Fe-S] clusters by the microbial protein YtfE remain poorly understood. The X-ray crystal structure of YtfE, in combination with EPR, magnetic circular dichroism (MCD), UV, and (17) O-labeling electron spin echo envelope modulation measurements, show that each iron of the oxo-bridged Fe(II) -Fe(III) diiron core is coordinatively unsaturated with each iron bound to two bridging carboxylates and two terminal histidines in addition to an oxo-bridge. Structural analysis reveals that there are two solvent-accessible tunnels, both of which converge to the diiron center and are critical for capturing substrates.
View Article and Find Full Text PDFEffect of transglutaminase (TGM) and ascorbic acid (AA) on composite sweet potato - wheat dough functional and rheological properties was studied. Partial substitution of wheat flour with sweet potato flour at the level of 20 % significantly (P ≤ 0.05) reduced glutenin, gliadin, dough stability, protein weakening, storage modulus (G') and viscous modulus (G″).
View Article and Find Full Text PDFORL J Otorhinolaryngol Relat Spec
September 2016
Objective: To investigate the source of snoring sound in patients with simple snoring (SS) and different degrees of obstructive sleep apnea syndrome (OSAS) in order to provide a basis for the surgical treatment of snoring.
Methods: Fifty-two patients with either SS or OSAS (with an apnea-hypopnea index ≤40) underwent drug-induced sleep nasendoscopy (DISN). Vibration sites in the pharyngeal cavity were observed.
Zhonghua Yi Xue Za Zhi
October 2013
Objective: To explore the efficacies of nasal cavity enlarging surgery in obstructive sleep apnea hypopnea syndrome (OSAHS) patients with nasal obstruction.
Methods: From April 2011 to October 2012, a total of 22 OSAHS patient with nasal obstruction underwent nasal cavity enlargement. Natural overnight snoring was digitally recorded, polysomnography (PSG) performed simultaneously and nasal resistance measured at pre- and post-operation.
Background: Pen c 13, identified as a 33-kDa alkaline serine protease, is a major allergen secreted by Penicillium citrinum. Detailed knowledge about the epitopes responsible for IgE binding would help inform the diagnosis/prognosis of fungal allergy and facilitate the rational design of hypoallergenic candidate vaccines. The goal of the present study was to characterize the IgE epitopes of Pen c 13.
View Article and Find Full Text PDFCobra CTX A3, the major cardiotoxin (CTX) from Naja atra, is a cytotoxic, basic β-sheet polypeptide that is known to induce a transient membrane leakage of cardiomyocytes through a sulfatide-dependent CTX membrane pore formation and internalization mechanism. The molecular specificity of CTX A3-sulfatide interaction at atomic levels has also been shown by both nuclear magnetic resonance (NMR) and X-ray diffraction techniques to reveal a role of CTX-induced sulfatide conformational changes for CTX A3 binding and dimer formation. In this study, we investigate the role of sulfatide lipid domains in CTX pore formation by various biophysical methods, including fluorescence imaging and atomic force microscopy, and suggest an important role of liquid-disordered (ld) and solid-ordered (so) phase boundary in lipid domains to facilitate the process.
View Article and Find Full Text PDFZhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
August 2011
Objective: To observe the distribution of Gd-DTPA in the inner ear of guinea pig by MRI at different time points after intratympanic administration, explore the optical time for observing the whole inner ear. To study the pharmacokinetic feature of Gd-DTPA in the inner ear, and find out whether discrimination of endolymph and perilymph can be obtained under current conditions.
Methods: Sixty-five guinea pigs were randomly divided into 13 groups, after diluted Gd-DTPA intratympanic injection, each group of guinea pigs were scanned through MRI (3D-T1 FSE sequence) at different time points (0 h, 0.
This manuscript describes the interaction of low-molecular-weight DNICs with short peptides designed to explore the stability and structure of DNIC-peptide/RRE-peptide constructs. Although characterization of protein-bound and low-molecular-weight DNICs is possible via EPR, XAS, and NRVS, this study demonstrates that the combination of aqueous IR ν(NO) and UV-vis spectra can serve as an efficient tool to characterize and discriminate peptide-bound DNICs and RREs. The de novo chelate-cysteine-containing peptides KC(A)(n)CK-bound (n = 1-4) dinitrosyliron complexes KC(A)(n)CK-DNIC (CnA-DNIC) and monodentate-cysteine-containing peptides KCAAK-/KCAAHK-bound Roussin's red esters (RREs) KCAAK-RRE/KCAAHK-RRE were synthesized and characterized by aqueous IR, UV-vis, EPR, CD, XAS, and ESI-MS.
View Article and Find Full Text PDFThe hydrolytic activity of secretory phospholipase A(2) (PLA(2)) is regulated by many factors, including the physical state of substrate aggregates and the chemical nature of phospholipid molecules. In order to achieve strong binding of PLA(2) on its substrates, many previous works have used anionic lipid dispersion to characterize the orientation and penetration depth of PLA(2) molecules on membrane surfaces. In this study, we applied monolayer technique with controllable surface area to investigate the PLA(2)s of Taiwan cobra venom and bee venom on zwitterionic phophatidylcholine monolayers and demonstrated an optimum hydrolytic activity at a surface pressure of 18 and 24 mN/m, respectively.
View Article and Find Full Text PDFZhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
April 2010
Objective: To visualize the endolymph in Meniere's disease by applying for non-invasive intratympanic gadolinium through eustachian tube and three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (3D-FLAIR MRI).
Methods: With a 3 Tesla magnetic resonance imaging (MRI) unit, 3D-FLAIR imaging was performed 24 h after intratympanic gadolinium through eustachian tube in seven patients with Meniere's disease. Pure tone test was performed 24 h before and after administered gadolinium.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
August 2009
Objective: To describe a series of patients with multiple canal involvement in benign paroxysmal positional vertigo (BPPV), with respect to diagnosis and management.
Methods: Ninety-five individuals with symptoms of BPPV and positional nystagmus were included in this study. The diagnosis was based on a history of brief episodes of vertigo and the presence of multiple positional nystagmus as confirmed by video-oculographic examination.
The water-soluble Roussin's red ester [(NO)(2)Fe(mu-SCH(2)CH(2)P(O)(CH(2)OH)(2))(2)Fe(NO)(2)] (1), a potential photochemical prodrug of an NO precursor, was synthesized from the reaction of HSCH(2)CH(2)P(O)(CH(2)OH)(2) (F) and [Fe(CO)(2)(NO)(2)]. The IR v(NO) stretching frequencies of complex 1 appear at 1759 (s), 1784 (s) and 1816 (w) cm(-1) in buffer (pH = 7.4).
View Article and Find Full Text PDFZhongguo Yi Xue Ke Xue Yuan Xue Bao
December 2008
Objective: To explore the normal values of vestibular autorotation test (VAT) in young people.
Methods: VAT was performed in 31 young people aged 20-30 years. The measured value were analyzed and compared with the reference normal value.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao
December 2008
Objective: To attempt to visualize the endolymph in patients with Meniere's disease by applying non-invasive intratympanic gadolinium through eustachian tube and three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (3D-FLAIR MRI).
Methods: With a 3 Tesla magnetic resonance imaging (MRI) unit, 3D-FLAIR imaging was performed 24 hours after intratympanic gadolinium through eustachian tube in two patients with medically active and intractable Meniere's disease. Pure tone test and tympanometry were performed 24 hours before and after the administration of gadolinium.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao
December 2008
Objective: To evaluate the pathological status of the vestibular system associated with noise-induced inner ear impairment.
Methods: Totally 68 patients with noise-induced hearing loss (NIHL) who presented with vestibular symptoms including vertigo, dizziness, and chronic disequilibrium were screened. All patients underwent a series of conventional vestibular function tests and vestibular autorotation test and the results were retrospectively reviewed and evaluated.
Previous nanoscale investigations of the gel-state membrane surface structure under the action of phospholipase A(2) (PLA(2)) suggest that single enzymes at work scoot on the membrane surface from the observed defects, which creates nanosized channels oriented along the lipid crystal-packing structure. To date, however, there have been no reports of direct observation of PLA(2) at the single-molecule level focusing on how the enzymes interact with the defects. Herein, we report a single-molecule fluorescence microscopy study on the action of enzymatically active rhodamine B-labeled cobra PLA(2) on a supported lipid membrane with visible packing defects on a glass substrate.
View Article and Find Full Text PDFThe major cardiotoxin from Taiwan cobra (CTX A3) is a pore forming beta-sheet polypeptide that requires sulfatide (sulfogalactosylceramide, SGC) on the plasma membrane of cardiomyocytes for CTX-induced membrane leakage and cell internalization. Herein, we demonstrate by fluorescence spectroscopic studies that sulfatides induce CTX A3 oligomerization in sulfatide containing phosphatidylcholine (PC) vesicles to form transient pores with pore size and lifetime in the range of about 30 A and 10(-2) s, respectively. These values are consistent with the CTX A3-induced conductance and mean lifetime determined previously by using patch-clamp electrophysiological experiments on the plasma membrane of H9C2 cells.
View Article and Find Full Text PDFZhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
June 2007
Objective: To analyse the video-oculographic findings of positional tests and evaluate the efficacy of canalith repositioning procedure (CRP) in patients with paroxysmal positional vertigo ( BPPV) of the anterior semicircular canal (ASC).
Methods: A retrospective study of 31 patients with ASC BPPV. Then the CRP was performed.
Cobra cardiotoxins (CTXs) are three-fingered polypeptides with positively charged domains that have been shown to bind to anionic ligands of snake venom citrate, glycosaminoglycans, sulfoglycosphingolipid, and nucleotide triphosphate with various biochemical effects including toxin dimerization, cell surface retention, membrane pore formation, cell internalization and blocking of enzymatic activities of kinase and ATPase. The reported anionic binding sites, however, are found to be different among different CTX homologues for potentially different CTX activities. Herein, by NMR studies of the binding of inorganic phosphate, dATP (stable form of ATP), and heparin-derived tetrasaccharide to Naja atra CTX A1, a novel CTX molecule exhibiting in vivo necrotic activity on skeletal muscle, we demonstrate that diverse ligands binding to CTXs could also occur at a single protein site with flexible side chain interactions.
View Article and Find Full Text PDFZhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
March 2007
Objective: To investigate the short-term efficacy and safety of transtympanic pressure treatment in the management of recalcitrant vertigo in Meniere disease in order to lead to the further long-term treatment.
Methods: Using cross-sectional case study, eighteen patients with medically intractable and active Meniere disease were opted to manage with Meniett pulse generator. A standard ventilation tube was inserted in the affected ear and the treatment period with Meniett pulse generator was 8 weeks.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
October 2006
Objective: To study human vestibular cerebral representations by combining right-sided ice-water stimulation at 0 degree C with blood oxygenation level dependent functional magnetic resonance imaging (BOLD-fMRI) and to evaluate the value of this method in the functional localization of human vestibular cortex.
Methods: Twenty right-handed volunteers (12 men and 8 women) received unilateral irrigation of the right external auditory meatu for 15 s with 15 ml of water at 0 degrees C during fMRI in complete darkness. The functional imaging of brain cortex was acquired with a 1.
Human pancreatitis-associated protein was identified in pathognomonic lesions of Alzheimer disease, a disease characterized by the presence of filamentous protein aggregates. Here, we showed that at physiological pH, human pancreatitis-associated protein forms non-Congo Red-binding, proteinase K-resistant fibrillar aggregates with diameters from 6 up to as large as 68 nm. Interestingly, circular dichroism and Fourier transform infrared spectra showed that, unlike typical amyloid fibrils, which have a cross-beta-sheet structure, these aggregates have a very similar secondary structure to that of the native protein, which is composed of two alpha-helices and eight beta-strands, as determined by NMR techniques.
View Article and Find Full Text PDFTo plants, copper is vitally essential at low concentrations but extremely toxic at elevated concentrations. Plants have evolved a suite of mechanisms that modulate the uptake, distribution, and utilization of copper ions. These mechanisms require copper-interacting proteins for transporting, chelating, and sequestrating copper ions.
View Article and Find Full Text PDFZhonghua Liu Xing Bing Xue Za Zhi
September 2005
Objective: To understand the various factors causing vertigo and balance disorders in the elderly.
Methods: 118 elderly patients (aged equal or older than 60 years of age) with vertigo or balance disorders were retrospectively analyzed through clinical symptoms, audio-vestibular function tests, X-ray, CT scan or MRI in cervical vertebras, brain and inner ears, ultrasonography, transcranial doppler (TCD) or magnetic resonance angiography (MRA) in blood vessels on head and neck.
Results: Of 118 patients, 70 (23%) of them suffered perip heral vestibular disorders while 29 (58%) having cerebral vertigo or dizzness, leaving 19 cases (16%) as unclassified.