• Title/Summary/Keyword: retardation function

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Structure-Function Analysis of DNA Binding Domain of the Yeast ABF1 Protein (효모 ABF1 단백질의 DNA Binding 부위에 대한 구조 기능 연구)

  • Cho, Gi-Nam;Lee, Sang-Kyung;Kim, Hong-Tae;Kim, Ji-Young;Rho, Hyune-Mo;Jung, Gu-Hung
    • Korean Journal of Microbiology
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    • v.32 no.2
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    • pp.102-108
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    • 1994
  • Autonomously replicating sequence Binding Factor 1(ABF1) is a DNA-binding protein that specifically recognizes the $RTCRYN_5ACG$ at many sites in the yeast genome including the promoter element, mating-type silencer and ARS. To express the intact full-length ABF1 gene in E. coli, the ABF1 gene has been cloned into pMAL-c2 and His-61, Leu-353 and Leu-360 were substituted with other amino acid. ABF1 fusion proteins of wild type ABF1 and H61A, L353R and L360R nutants were purified by amylose resin affinity chromatography. Fusion protein of MBP and ABF1 was digested by Factor Xa and Characterized by gel retardation assay and complementation test. As aresult, we suggested that other DNA binding motif except atypical inc-finger motif is in the middle region of ABF1.

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Neuronal function and dysfunction of CYFIP2: from actin dynamics to early infantile epileptic encephalopathy

  • Zhang, Yinhua;Lee, Yeunkum;Han, Kihoon
    • BMB Reports
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    • v.52 no.5
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    • pp.304-311
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    • 2019
  • The cytoplasmic FMR1-interacting protein family (CYFIP1 and CYFIP2) are evolutionarily conserved proteins originally identified as binding partners of the fragile X mental retardation protein (FMRP), a messenger RNA (mRNA)-binding protein whose loss causes the fragile X syndrome. Moreover, CYFIP is a key component of the heteropentameric WAVE regulatory complex (WRC), a critical regulator of neuronal actin dynamics. Therefore, CYFIP may play key roles in regulating both mRNA translation and actin polymerization, which are critically involved in proper neuronal development and function. Nevertheless, compared to CYFIP1, neuronal function and dysfunction of CYFIP2 remain largely unknown, possibly due to the relatively less well established association between CYFIP2 and brain disorders. Despite high amino acid sequence homology between CYFIP1 and CYFIP2, several in vitro and animal model studies have suggested that CYFIP2 has some unique neuronal functions distinct from those of CYFIP1. Furthermore, recent whole-exome sequencing studies identified de novo hot spot variants of CYFIP2 in patients with early infantile epileptic encephalopathy (EIEE), clearly implicating CYFIP2 dysfunction in neurological disorders. In this review, we highlight these recent investigations into the neuronal function and dysfunction of CYFIP2, and also discuss several key questions remaining about this intriguing neuronal protein.

The Effect of the Bobath Approach on Balance and Motor Ability in Mentally Retarded Child (보바스 접근방법이 정신지체 아동의 균형 및 운동능력에 미치는 영향: 단일사례연구)

  • Ro, Hyo-Lyun
    • Journal of Korean Physical Therapy Science
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    • v.15 no.2
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    • pp.79-86
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    • 2008
  • Background: The purpose of this study was to present a practical method of medical treatment to improve the balance and motor ability of the mentally retarded child with a single mentally retarded child-subject. Methods: The subject of the study was a 39-month-old mentally retarded female. This study included a 2-week basic period and a 13-week treatment period. The treatment method was based on the Bobath Approach. Gross motor function measurement (GMFM) was used to examine changes in motor ability, and the Pediatric Balance Scale (PBS) was used to measure changes in balance ability. The curative program was composed of normalization of muscle tone, strengthening of leg endurance and muscular strength, the improvement of trunk alignment, and the increase of balance. Visual rate of change was used to examine the results. Results: As a result of this study, balance ability increased on the Pediatric Balance Scale (PBS) by 24 points, and motor function increased in terms of Gross Motor Function Measurement (GMFM) by 6.9% (18 points). Standing increased by 41% (16 points), and walking, running, and jumping increased by 31.9% (23 points) compared to thebasic period. Therefore, the Bobath Approach appears to be an appropriate method to improve balance and motor ability in mentally retarded children. Conclusion: It is surmised that aggressive intervention by physical therapists and occupational therapists, and a follow-up study, are required for the growth of motor ability in mentally retarded children.

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Rheological Properties of Rough Rice (II) -Compressive Creep of Rough Rice Kernel- (벼의 리올러지 특성(特性)(II) -곡립(穀粒)의 압축(壓縮)크리이프-)

  • Kim, M.S.;Kim, S.R.;Park, J.M.
    • Journal of Biosystems Engineering
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    • v.15 no.3
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    • pp.219-229
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    • 1990
  • The compression creep behavior of grains when loaded depends not only on load but also on duration of load application. The most common methods of studying the load-time characteristics of agricultural products is by employing rheological models such as Burger's model. However it is sometimes not sufficient to describe the viscoelastic behavior of grains to be Burger's model. For this reason, this study was conducted to develop the rheological model which represented the creep compliance response of the rough rice kernel and was a function of initial stress applied and time. The effects of the initial stress applied and the moisture content on the compression creep behavior of the rough rice kernel were analyzed. The results were obtained from the study as follows: 1. Since the viscoelastic behavior of the rough rice kernel was nonlinear, the transient and steady state creep compliance was satisfactorily modelled as follows: $$J({\sigma},t)=A{\sigma}^B[C+Dt-exp(-Ft)]$$ But, for the every stress applied, the compression creep behavior of the samples tested can be well described by Burger's model respectively. 2. The creep compliance, the instantaneous elastic strain, the retarded elastic strain and the viscous strain of the sample tested generally increased in magnitude with increasing the applied initial stress and the moisture content used in the tests. At low moisture content, the creep compliance for the Japonica-type rough rice kernel Was a little higher than those for Indica-type and at high moisture content, vice versa at high moisture content. 3. The retardation times of the samples had not an uniform tendency by the initial stress and the moisture content. The retardation times ranged from 0.66 to 6.76 seconds, and the creep progressed from transient to steady state at a relatively high rate. 4. The less viscous strain than the instantaneous elastic strain for the samples tested indicated that rough rice kernel behaved as a viscoelastic body characterized by elasticity than viscosity.

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Effect of Streptozotocin-Induced Diabetes on Bone and Heart Development in Juvenile Rats

  • Kim, Joo-Heon;Lee, Young-Jeon;Lee, Sang-Un;Suzuki, Takao;Lee, Sang-Kil;Kang, Tae-Young;Hong, Yong-Geun
    • Reproductive and Developmental Biology
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    • v.34 no.2
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    • pp.81-88
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    • 2010
  • Our objective of current study was to investigate the development of bone and heart in association with diabetes mellitus (DM). DM was induced by administering an intraperitoneal injection of streptozotocin (STZ; 60 mg/kg) to 4-week-old Sprague-Dawley rats. Body weight and blood glucose were monitored, and rats were sacrificed after 2 or 5 weeks. The left ventricle (LV), including the interventricular septum, was weighed, and body weight and tibial bone length were assessed. Young diabetic rats showed reduced growth in terms of tibial length and body weight compared to controls. Moreover, diabetic males showed more significant growth suppression and reduced LV size than diabetic females. Morphometric analysis of tibiae from diabetic rats revealed suppressed bone growth at 2 and 5 weeks, with no difference between genders. STZ-induced diabetes decreased bone growth and retarded pre-pubertal heart development. As a result, diabetes may increase cardiovascular risk factors and lead to eventual heart failure. Therefore, new therapeutic approaches are required for diabetic children exhibiting growth retardation. Heart growth factor, exercise, and cardiopulmonary physical therapy may be required to promote heart development and physiological function.

Function of mORF1 Protein as a Terminal Recognition Factor for the Linear Mitochondrial Plasmid pMLP1 from Pleurotus ostreatus

  • Kim, Eun-Kyoung;Roe, Jung-Hye
    • Journal of Microbiology
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    • v.37 no.4
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    • pp.229-233
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    • 1999
  • The mitochondrial plasmid pMLP1 from a white-rot fungus, Pleurotus ostreatus, is a double-stranded DNA containing 381 bp terminal inverted repeat (TIR) whose 5'-ends are covalently bound by terminal proteins. The plasmid contains two major open reading frames (ORFs), encoding putative DNA and RNA polymerases, and a minor ORF encoding a small, highly basic protein. To identify the DNA binding activity that recognizes the TIR region of pMLP1, gel retardation assays were performed with mitochondrial extracts. A specific protein binding to a region between 123 and 248 nt within TIR was observed. We examined whether the gene product of mORF1 bindes to this region specifically. E. coli cell extract which contains an overproduced mORF1 protein formed a complex specific to the region between 123 and 248 nt. Inclusion of mORF1 protein in the specific complex formed between P. ostreatus mitochondrial extract and TIR was confirmed by a supershift assay using polyclonal antibodies against the mORF1 protein. Our result suggest that the product of mORF1 may function as a terminal region recognition factor (TRF), recognizing an internal region in TIR.

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Angiotensin receptor blocker induced fetopathy: two case reports and literature review

  • Jinwoon Joung;Heeyeon Cho
    • Childhood Kidney Diseases
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    • v.27 no.2
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    • pp.121-126
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    • 2023
  • The administration of angiotensin type 2 receptor blockers (ARBs) during pregnancy is known to cause ARB fetopathy, including renal insufficiency. We aimed to analyze the outcomes of two patients who survived ARB fetopathy and perform an accompanying literature review. Case 1 was exposed antenatally from a gestational age of 30 weeks to valsartan because of maternal pregnancy-induced hypertension. The patient presented with oliguria immediately after birth, and renal replacement therapy was administered for 24 days. Seven years after birth, renal function was indicative of stage 2 chronic kidney disease (CKD) with impaired urinary concentration. Case 2 had a maternal history of hypertension and transient ischemic attack and was treated with olmesartan until 30 weeks of pregnancy. Renal replacement therapy was performed for 4 days since birth. After 8 years, the patient is with CKD stage 2, with intact tubular function. Recent reports suggest that ARB fetopathy might manifest as renal tubular dysgenesis and nephrogenic diabetes insipidus, in contrast to mild alterations of glomerular filtration. Tubular dysfunction may induce CKD progression and growth retardation. Patients with ARB fetopathy should be monitored until adulthood. The ARB exposure period might be a critical factor in determining the severity and manifestations of fetopathy.

Ataxin-2 Dysregulation Triggers a Compensatory Fragile X Mental Retardation Protein Decrease in Drosophila C4da Neurons

  • Cha, In Jun;Lee, Davin;Park, Sung Soon;Chung, Chang Geon;Kim, Seung Yeon;Jo, Min Gu;Kim, Seung Yeol;Lee, Byung-Hoon;Lee, Young-Sam;Lee, Sung Bae
    • Molecules and Cells
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    • v.43 no.10
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    • pp.870-879
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    • 2020
  • Dendrites require precise and timely delivery of protein substrates to distal areas to ensure the correct morphology and function of neurons. Many of these protein substrates are supplied in the form of ribonucleoprotein (RNP) complex consisting of RNA-binding proteins (RBPs) and mRNAs, which are subsequently translated in distal dendritic areas. It remains elusive, however, whether key RBPs supply mRNA according to local demands individually or in a coordinated manner. In this study, we investigated how Drosophila sensory neurons respond to the dysregulation of a disease-associated RBP, Ataxin-2 (ATX2), which leads to dendritic defects. We found that ATX2 plays a crucial role in spacing dendritic branches for the optimal dendritic receptive fields in Drosophila class IV dendritic arborization (C4da) neurons, where both expression level and subcellular location of ATX2 contribute significantly to this effect. We showed that translational upregulation through the expression of eukaryotic translation initiation factor 4E (eIF4E) further enhanced the ATX2-induced dendritic phenotypes. Additionally, we found that the expression level of another disease-associated RBP, fragile X mental retardation protein (FMRP), decreased in both cell bodies and dendrites when neurons were faced with aberrant upregulation of ATX2. Finally, we revealed that the PAM2 motif of ATX2, which mediates its interaction with poly(A)-binding protein (PABP), is potentially necessary for the decrease of FMRP in certain neuronal stress conditions. Collectively, our data suggest that dysregulation of RBPs triggers a compensatory regulation of other functionally-overlapping RBPs to minimize RBP dysregulation-associated aberrations that hinder neuronal homeostasis in dendrites.

Sequence Analysis and Functional Expression of the Structural and ]Regulatory Genes for Pyruvate Dehydrogenase

  • Hwan Youn;Jangyul Kwak
    • Journal of Microbiology
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    • v.40 no.1
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    • pp.43-50
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    • 2002
  • A cluster of genes encoding the pyruvate dehydrogenase complex (PDC) of Streptomyces seoulensis, a Gram-positive bacterium, was cloned and sequenced. The genes of S. seoulensis consist of four open reading frames. The first gene, lpd, which encodes a lipoamide dehydrogenase, is followed by pdhB encoding a dihydrolipoamide acetyltransferase (E2p), pdhR, a regulatory gene, and pdhA encoding a pyruvate dehydrogenase component (Elp). Elp had an unusual homodimeric subunit, which has been known only in Gram-negative bacteria S. seoulensis E2p contains two lipoyl domains like those of humans and Streptomyces faecalis. The pdhR gene appears to be clustered with the structural genes of S. seoulensis PDC. The PdhR-overexpressed S. seoulensis howed growth retardation and the decrease of Elp, indicating that PdhR regulates the function of PDC by repressing the expression of Elp. A strain of Streptomyces licidans overexpressing S. seoulensis PdhR showed a significant decreasein the level of actinorhodin, implying a regulatory role for Streptomyces PDC in antibiotic biosynthesis.

Metabolism of Very Long-Chain Fatty Acids: Genes and Pathophysiology

  • Sassa, Takayuki;Kihara, Akio
    • Biomolecules & Therapeutics
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    • v.22 no.2
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    • pp.83-92
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    • 2014
  • Fatty acids (FAs) are highly diverse in terms of carbon (C) chain-length and number of double bonds. FAs with C>20 are called very long-chain fatty acids (VLCFAs). VLCFAs are found not only as constituents of cellular lipids such as sphingolipids and glycerophospholipids but also as precursors of lipid mediators. Our understanding on the function of VLCFAs is growing in parallel with the identification of enzymes involved in VLCFA synthesis or degradation. A variety of inherited diseases, such as ichthyosis, macular degeneration, myopathy, mental retardation, and demyelination, are caused by mutations in the genes encoding VLCFA metabolizing enzymes. In this review, we describe mammalian VLCFAs by highlighting their tissue distribution and metabolic pathways, and we discuss responsible genes and enzymes with reference to their roles in pathophysiology.