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Craniovertebral Junction Abnormalities in Surgical Patients With Congenital Muscular Torticollis
Ahn, Ah-Reum,Rah, Ueon Woo,Woo, Ji-Eun,Park, Sunghoon,Kim, Sanghyun,Yim, Shin-Young by Mutaz B. Habal, MD. 2018 JOURNAL OF CRANIOFACIAL SURGERY - Vol.29 No.3
ABSTRACT: Our clinical experience led us to realize that craniovertebral junction (CVJ) abnormalities were common in surgical patients with congenital muscular torticollis (CMT). This study aimed to report the concurrence rate of CVJ abnormalities in surgical patients with CMT, along with comprehensive evaluation of type of concurrent CVJ abnormalities. This was a retrospective cohort study in a tertiary hospital, including 41 subjects who underwent surgical release for CMT at the mean age of 8.38 years. The presence of CVJ abnormalities was analyzed, using craniofacial 3-dimensional computed tomography images. The concurrence rate of CVJ abnormalities was 70% in surgical patients with CMT. Subjects with CVJ abnormalities had, on average, 1.48 abnormalities. The CVJ abnormalities were rotation and lateral shift of the atlanto-axial joint along with rotation of atlanto-occipital joint, where rotation of the atlanto-axial joint was most common (82.76%). There is no patient with anterior shift of the atlanto-axial joint. Chronic mechanical tension by the contracted unilateral sternocleidomastoid muscle of CMT could be responsible for concurrent CVJ abnormalities. The CVJ abnormalities are more common in the atlanto-axial joint than in the atlanto-occipital joint. In conclusions, CVJ abnormality seems to be a common concurrent skeletal complication of CMT, at least, in surgical patients. The CVJ abnormality might be included in the list of skeletal complications of CMT. If CVJ abnormalities are significantly more common in surgical patients with CMT, CVJ abnormalities might be one of predictors of surgical patients with CMT.
Imidazole Trimer-Water Complexes in Superfluid Helium Nanodroplets: Water Stretching Modes
Ahn, Ah-Reum,Lee, Seung-Jun,Lee, Seul-Ki,Min, Ah-Reum,Kim, Yu-Sic,Jung, Hyeon-Jin,Hong, Seong-Min,Lee, Joon-Hwa,Choi, Myong-Yong,Miller, Roger E. Korean Chemical Society 2011 Bulletin of the Korean Chemical Society Vol.32 No.4
Ahn, Jong Hyeon,Kim, Ah Reum,Kim, Nayoung K. D.,Park, Woong-Yang,Kim, Ji Sun,Kim, Minkyeong,Park, Jongkyu,Lee, Jung-Il,Cho, Jin Whan,Cho, Kyung Rae,Youn, Jinyoung The Korean Movement Disorder Society 2019 Journal of movement disorders: official journal of Vol.12 No.2
<P><B>Objective</B></P><P>The aim of this study was to investigate the efficacy of globus pallidus interna deep brain stimulation (GPi-DBS) for treating dystonia due to the <I>GNAL</I> mutation.</P><P><B>Methods</B></P><P>We provide the first report of a dystonia patient with a genetically confirmed <I>GNAL</I> mutation in the Korean population and reviewed the literature on patients with the <I>GNAL</I> mutation who underwent GPi-DBS. We compared the effectiveness of DBS in patients with the <I>GNAL</I> mutation compared to that in patients with DYT1 and DYT6 in a previous study.</P><P><B>Results</B></P><P>Patients with the <I>GNAL</I> mutation and those with DYT1 had higher early responder rates (<I>GNAL</I>, 5/5, 100%; DYT1, 7/7, 100%) than did patients with DYT6 (<I>p</I> = 0.047). The responder rates at late follow-up did not differ statistically among the three groups (<I>p</I> = 0.278). The decrease in the dystonia motor scale score in the <I>GNAL</I> group was 46.9% at early follow-up and 63.4% at late follow-up.</P><P><B>Conclusion</B></P><P>GPi-DBS would be an effective treatment option for dystonia patients with the <I>GNAL</I> mutation who are resistant to medication or botulinum toxin treatment.</P>
( Ah Reum Park ),( Jeong Hoon Park ),( Hye Jin Ahn ),( Ji Yeon Jang ),( Byung Jo Yu ),( Byung Hwan Um ),( Jeong Jun Yoon ) 한국균학회 2015 韓國菌學會誌 Vol.43 No.1
β-Glucosidase, which hydrolyzes cellobiose into two glucoses, plays an important role in the process of saccharification of the lignocellulosic biomass. In this study, we optimized the activity of β-glucosidase of brown-rot fungus Fomitopsis pinicola KCTC 6208 using the response surface methodology (RSM) with various concentrations of glucose, yeast extract and ascorbic acid, which are the most significant nutrients for activity of β-glucosidase. The highest activity of β-glucosidase was achieved 3.02% of glucose, 4.35% of yeast extract, and 7.41% ascorbic acid where ascorbic acid was most effective. The maximum activity of β-glucosidase predicted by the RSM was 15.34 U/mg, which was similar to the experimental value 14.90 U/mg at the 16th day of incubation. This optimized activity of β-glucosidase was 23.6 times higher than the preliminary activity value, 0.63 U/mg, and was also much higher than previous values reported in other fungi strains. Therefore, a simplified medium supplemented with a cheap vitamin source, such as ascorbic acid, could be a cost effective mean of increasing β-glucosidase activity.
Ahn, Bo-Eun,Cha, Joonseok,Lee, Eun-Jin,Han, Ah-Reum,Thompson, Charles J.,Roe, Jung-Hye Blackwell Science Ltd 2006 Molecular microbiology Vol.59 No.6
<P>Summary</P><P>Nickel serves as a cofactor for various microbial enzymes including superoxide dismutase (SOD) found in <I>Streptomyces</I> spp. In <I>Streptomyces coelicolor</I>, nickel represses and induces production of Fe-containing and Ni-containing SODs, respectively, primarily at the transcriptional level. We identified the nickel-responsive regulator (Nur), a Fur (<U>f</U>erric-<U>u</U>ptake <U>r</U>egulator) homologue, which binds to the promoter region of the <I>sodF</I> gene encoding FeSOD in the presence of nickel. Disruption of the <I>nur</I> gene caused constitutive expression of FeSOD and no induction of NiSOD in the presence of nickel. The intracellular level of nickel was higher in a <I>Δnur</I> mutant than in the wild type, suggesting that Nur also regulates nickel uptake in <I>S. coelicolor</I>. A putative nickel-transporter gene cluster (<I>nikABCDE</I>) was identified in the genome database. Its transcription was negatively regulated by Nur in the presence of nickel. Purified Nur protein bound to the <I>nikA</I> promoter region in a nickel-dependent way. These results support the action of Nur as a regulator of nickel homeostasis and antioxidative response in <I>S. coelicolor</I>, and add a novel nickel-responsive member to the list of versatile metal-specific regulators of the Fur family.</P>
Successful Pallidal Stimulation in a Patient with KMT2B-Related Dystonia
문준규,Ah Reum Kim,Jong Hyeon Ahn,Minkyeong Kim,Jin Whan Cho,이정일,조경래,Jinyoung Youn 대한파킨슨병및이상운동질환학회 2020 Journal Of Movement Disorders Vol.13 No.2
Although the KMT2B gene was identified as a causative gene for early-onset generalized dystonia, the efficacy of deep brain stimulation(DBS) in KMT2B-related dystonia has not been clearly elucidated. Here, we describe a 28-year-old woman who developedgeneralized dystonia with developmental delay, microcephaly, short stature, and cognitive decline. She was diagnosed with KMT2B-related dystonia using whole-exome sequencing with a heterozygous frameshift insertion of c.515dupC (p.T172fs) in theKMT2B gene. Oral medications and botulinum toxin injection were not effective. The dystonia markedly improved with bilateralpallidal DBS (the Burke-Fahn-Marsden Dystonia Rating Scale score was reduced from 30 to 5 on the dystonia movement scaleand from 11 to 1 on the disability scale), and she could walk independently. From this case, we suggest that bilateral globus pallidusinternus DBS can be an effective treatment option for patients with KMT2B-related generalized dystonia.
Kang, Ah-Reum,Ahn, Sung-Won,Lee, Seong-Jae,Lee, Byung-Hwan,Lee, Sung-Sik,Kim, Ju-Min 한국유변학회 2011 Korea-Australia rheology journal Vol.23 No.4
Medium viscoelasticity contributes to the lateral particle migration and particle segregation under channel flows. It is recently reported that the equilibrium positions of the particles in dilute suspensions are greatly affected in a rectangular microchannel by the relative ratio of the medium viscoelastic and inertial forces (Yang et al., 2011). Here, we summarize the forces which may exert on the particles in concentrated suspensions under the viscoelastic flows including inertial lift force, and the shear-induced migration effect arising from particle-particle interaction. In addition, we report some preliminary results regarding particle segregation in concentrated suspensions (i.e., ${\phi}_{bulk}=0.05$, 0.1) under both inertial and inertialess viscoelastic flows in the rectangular microchannel. Similar to our previous results in dilute suspensions (Yang et al., 2011), the particles tend to migrate toward low first normal stress regions, and the particle migration is greatly affected by existence of inertia. We firstly report the formation of multi-layered particle trains along the corners in this work. These results give an insight that the medium viscoelascticity would contribute to the design of particle separation devices in complex suspensional flows (e.g. blood cell separation), and also the understanding of particle dynamics in confined environments under viscoelastic flows.