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Berlin Brandenburg


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  • 1
    Language: English
    In: Enzyme, 1987, Vol.38(1-4), pp.91-107
    Description: Our early study of isovaleric acidemia (IVA) indicated that isovaleryl-CoA is dehydrogenated by an enzyme that is specific for isovaleryl-CoA. We subsequently identified and purified isovaleryl-CoA dehydrogenase (IVD) and 2-methyl-branched chain acyl-CoA dehydrogenase, which were previously unknown. We also purified and characterized three previously known acyl-CoA dehydrogenases. Five acyl-CoA dehydrogenases share similar molecular features and reaction mechanisms, indicating a close evolutionary relationship. Using the tritium release assay and [(35)S]methionine labeling/immunoprecipitation, we showed that IVA is due to a mutation of IVD. We also demonstrated that there are at least 5 distinct forms of mutant IVD, indicating an extensive molecular heterogeneity. Furthermore, we cloned cDNAs encoding IVD and medium-chain acyl-CoA dehydrogenases. The comparison of their complete primary sequences revealed a high degree of homology, indicating that these enzymes belong to a gene family, the acyl-CoA dehydrogenase family.
    Keywords: Further Section ; Medium-Chain Acyl-Coa Dehydrogenase Deficiency ; Acyl-Coa Dehydrogenase Family ; In Vitro Translation ; Molecular Cloning ; Nucleotide Sequence ; Gene Mapping ; Isovaleric Acidemia ; Isovaleryl-Coa Dehydrogenase ; Medicine
    ISBN: 9783805547727
    ISBN: 3805547722
    ISSN: 0013-9432
    E-ISSN: 2504-2564
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  • 2
    Language: Japanese
    In: Nihon rinsho. Japanese journal of clinical medicine, September 2013, Vol.71(9), pp.1569-76
    Description: Human LPL is a glycoprotein enzyme with a molecular mass of 61 kDa, and it plays a key role in regulating the triglyceride (TG) levels in circulation by hydrolyzing TGs in TG-rich lipoproteins at the first step in their metabolism. Homozygous or compound heterozygous LPL deficiency causes severe fasting hypertriglyceridemia. Heterozygous LPL deficiency usually results in a normolipidemic state, but this may cause mild hypertriglyceridemia if heterozygotes are exposed to factors, such as high alcohol intake and/or a hyperinsulinemic state. Severe fasting hypertriglyceridemia is mainly caused by abnormalities of the LPL gene, whereas there are some cases caused by gene defects relating to synthesis and transport of LPL such as LMF and GPIHBP1, and by an autoantibody to LPL acting as inhibitor of LPL.
    Keywords: Pathology, Molecular ; Hypertriglyceridemia -- Genetics ; Lipoprotein Lipase -- Genetics ; Mutation -- Genetics
    ISSN: 0047-1852
    Source: MEDLINE/PubMed (U.S. National Library of Medicine)
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  • 3
    Language: English
    In: Journal of Radioanalytical and Nuclear Chemistry, 2013, Vol.296(1), pp.289-292
    Description: The adsorption behaviors of trivalent actinides and lanthanides on pyridine resin in lithium chloride aqueous solution were investigated. The adsorbed amounts of lanthanides and the degree of mutual separation of lanthanides increased with an increase in the concentration of lithium chloride in aqueous solution. The group separation of the trivalent actinides and lanthanides was observed. This separation phenomenon is similar in a hydrochloric acid solution. However, the adsorption behavior of lanthanides in lithium chloride is different from their behavior in a hydrochloric acid solution. This fact shows that the adsorption mechanisms of lanthanides in a lithium chloride aqueous solution and in a hydrochloric acid solution are different; the adsorption mechanisms are attributed to the ion exchange in a hydrochloric acid solution, and to the complex formation with pyridine group in a lithium chloride solution.
    Keywords: Trivalent actinides ; Lanthanide ; Lithium chloride aqueous solution ; Pyridine resin
    ISSN: 0236-5731
    E-ISSN: 1588-2780
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  • 4
    Language: Japanese
    In: 化学工学論文集, 2018, Vol.44(2), pp.91-93
    Description: 〈p〉筆者らは流脈の可視化に基づき,3S(Simple, Speedy, Stable)の性能を持つ新規な撹拌翼(HB翼)を開発した(Kato 〈i〉et al.〈/i〉, 2015a, 2015b).本報では,さらなるHB翼の実用化を目指し,マグネチックスターラーへの応用を試みた.通常用いられる円柱状のスターラーチップと比較した結果,種々の水溶液の混合に対し,大幅な混合時間の短縮を得た.〈/p〉
    Description: 〈p〉A new home base (HB) type impeller with 3S performance (simple, speedy and stable) was developed based on the streak line visualization method (Kato 〈i〉et al.〈/i〉, 2015a, 2015b). The HB impeller was employed as a magnetic stirrer for practical use in experimental processes. The mixing time of the HB impeller for various aqueous solutions was found to be shorter than that of a normal cylindrical stirring bar.〈/p〉
    Keywords: 混合 ; 撹拌 ; 新型翼 ; マグネチックスターラー ; Mixing ; Agitation ; New Type Impeller ; Magnetic Stirrer
    ISSN: 0386-216X
    E-ISSN: 13499203
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  • 5
    Language: English
    In: Journal of the Neurological Sciences, 15 January 2013, Vol.324(1-2), pp.109-112
    Description: To investigate acoustic function of Asidan/spinocerebellar ataxia type 36 (SCA36) in which sensorineural hearing loss may be found as one of extracerebellar symptom that can be a distinguishable feature from other degenerative ataxias. Acoustic function in the groups of normal control ( = 31), Asidan/SCA36 ( = 13), cortical cerebellar atrophy (CCA, = 28), multiple system atrophy of cerebellar predominance (MSA-C, = 48), SCA31 ( = 4), and other forms of SCAs ( = 14) was evaluated by pure tone average (PTA) calculated by the results of audiogram and brainstem auditory evoked potentials (BAEPs). PTA was significantly decreased in Asidan/SCA36 in comparison to normal control and other ataxic groups, but not significant within other ataxic groups and normal control. In comparison to other groups, Asidan/SCA36 showed a constant depression at 7 different frequencies in audiogram, especially at 4000 and 8000 Hz. BAEPs in 2 Asidan/SCA36 cases suggested possible involvement in the inner ear or the peripheral part of the auditory system. PTA in Asidan/SCA36 cases significantly correlated with their severity of ataxia. In addition to signs for motor neuron involvement, acoustic impairment in Asidan/SCA36 is another characteristic clinical feature that is distinguishable from other forms of SCAs.
    Keywords: Ataxia ; Motor Neuron Disease ; Audiogram ; Brainstem Auditory Evoked Potential ; Sca36 ; Asidan ; Ggcctg Repeat ; Nop56 ; Medicine
    ISSN: 0022-510X
    E-ISSN: 1878-5883
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  • 6
    Language: English
    In: International Journal of Chemical Engineering, 01 January 2018, Vol.2018
    Description: In recent years, a novel home base-type (HB-type) impeller was developed to be based on observation of the streak line pattern. An HB impeller must be simple, speedy, and stable (3S). When an HB impeller is used on the laboratory beaker scale, the mixing performance of the HB impeller is better...
    Keywords: Engineering
    ISSN: 1687-806X
    E-ISSN: 1687-8078
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  • 7
    Language: English
    In: Extremophiles, 2016, Vol.20(4), pp.415-424
    Description: An ionic liquid-tolerant bacterium, Bacillus amyloliquefaciens CMW1, was isolated from a Japanese fermented soybean paste. Strain CMW1 grew in the presence of 10 % (v/v) 1-butyl-3-methylimidazolium chloride ([BMIM]Cl), a commonly used ionic liquid. Additionally, strain CMW1 grew adequately in the presence of the hydrophilic ionic liquids 10 % (v/v) 1-ethyl-3-methylimidazolium trifluoromethanesulfonate ([EMIM]CF 3 SO 3 ) or 2.5 % (v/v) 1-butyl-3-methylimidazolium trifluoromethanesulfonate ([BMIM]CF 3 SO 3 ). Strain CMW1 produced an extracellular protease (BapIL) in the culture medium. BapIL was stable in the presence of 80 % (v/v) ionic liquids, [EMIM]CF 3 SO 3 , [BMIM]Cl, [BMIM]CF 3 SO 3 , 1-butyl-3-methylimidazolium tetrafluoroborate, 1-butyl-3-methylimidazolium hexafluorophosphate, and 1-butyl-3-methylimidazolium bis(trifluoromethylsulfonyl)imide, and functioned in 10 % (v/v) these ionic liquids. BapIL was stable at pH 4.0–12.6 or in 4004 mM NaCl solution, and exhibited activity in the presence of 50 % (v/v) hydrophilic or hydrophobic organic solvents. BapIL was completely inhibited by 1 mM PMSF and partially by 5 mM EDTA. BapIL belongs to the true subtilisins according to analysis of the deduced amino acid sequence. We showed that BapIL from the ionic liquid-tolerant B . amyloliquefaciens CMW1 exhibited tolerance to ionic liquid and halo, alkaline, and organic solvents.
    Keywords: Ionic Liquid ; Ionic liquid-tolerant protease ; . CMW1
    ISSN: 1431-0651
    E-ISSN: 1433-4909
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  • 8
    Language: English
    In: The journal of physical chemistry. B, 06 August 2009, Vol.113(31), pp.10619-27
    Description: We have investigated the relationship between the photophysical properties and structures of a series of directly linked zinc(II) porphyrin dimers (mmZ2, mbZ2, and bbZ2) using time-resolved spectroscopic measurements and theoretical calculations. Their unique characters such as CT nature and torsional motion are caused by interporphyrin interactions and steric effects, respectively, which can be fully understood in terms of three structural factors: linking position, dihedral angle, and linkage length. The orthogonal geometry and heterolinking of mmZ2 and mbZ2 induce the localized MOs and electron unbalance in the constituent porphyrin units, respectively, and consequently lead to distinct CT characters in spite of their different origin. On the other hand, a small interporphyrin steric hindrance in bbZ2 makes a torsional motion possible around the direct beta-beta' linkage in the excited state, which is correlated with the solvent dependence of the fast S(1) fluorescence decay component. On the basis of this work, we can gain further insight into the effect of individual structural factors on the photophysical properties, which provides a firm basis for further understanding of the photophysical properties mainly determined by the structural factors in multiporphyrin systems.
    Keywords: Porphyrins -- Optical Properties ; Porphyrins -- Structure ; Porphyrins -- Research ; Spectroscopy -- Usage ; Zinc Compounds -- Optical Properties ; Zinc Compounds -- Research;
    ISSN: 1520-6106
    E-ISSN: 15205207
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  • 9
    Language: English
    In: Proceedings of the National Academy of Sciences of the United States of America, 15 October 1985, Vol.82(20), pp.7081-7085
    Description: Variants of isovaleryl-CoA dehydrogenase (IVDHase, EC in 15 isovaleric acidemia fibroblast lines were analyzed using [ 35 S]methionine labeling, immunoprecipitation with anti-rat IVDHase antiserum, and NaDodSO 4 /polyacrylamide gel electrophoresis. Five distinct variants of IVDHase were detected. The molecular size of variant 1 (43 kDa) was indistinguishable from that of normal IVDHase (43 kDa), although the activity of this enzyme was as deficient (0-2.2% of normal control) as that of any other variant. It was synthesized as a precursor (45 kDa), which is the case for normal IVDHase. Variant 2 was synthesized as a 42-kDa precursor, but only a small portion of it was processed to the mature variant form (40 kDa). Variant 3 (41 kDa) was synthesized as a 43-kDa precursor. Variant 4 (40 kDa) was synthesized as a 42-kDa precursor that was readily processed to the mature form. In cells with variant 5, no material that crossreacted with the anti-rat IVDHase antibody was detected. These results suggest that variant 1 may be due to a point mutation, while variants 2-4 may be encoded by a different mutant IVDHase allele that causes the premature termination of translation, although other complex mechanisms are possible. A deletion, a nonsense mutation close to the NH 2 terminus or an extremely labile mRNA may give rise to variant 5.
    Keywords: Physical sciences -- Chemistry -- Chemical compounds ; Physical sciences -- Chemistry -- Chemical compounds ; Biological sciences -- Biology -- Cytology ; Biological sciences -- Biology -- Cytology ; Health sciences -- Medical sciences -- Immunology ; Biological sciences -- Biology -- Anatomy ; Biological sciences -- Biology -- Genetics ; Health sciences -- Medical sciences -- Immunology ; Applied sciences -- Materials science -- Materials ; Biological sciences -- Biology -- Genetics
    ISSN: 00278424
    E-ISSN: 10916490
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  • 10
    Language: English
    In: Optical Engineering, 05/01/2003, Vol.42(5), p.1249
    Description: Eine neue exakte Phasenanalyse und nachfolgende Phasenentzerrungsmethode wird für die dreidimensionale Gitterprojektions-Oberflächenprofilometrie eingesetzt. Dabei werden die frequenzmodulierten Gitter auf das Objekt projiziert und neun Rahmen mit einer CCD-Kamera mit der Phasenschrittmethode aufgenommen. Mit diesen neun Bildern werden die beiden Phasenkarten simultan untersucht. Die Phasenentzerrung wird für jedes Pixel unabhängig durchgeführt. Daher ist die vorgeschlagene Methode für Messungen komplex geformter Objekte mit großen Phasensprüngen geeignet. Mit der vorgeschlagenen Methode wurde eine horizontale Ebene bei verschiedenen Höhen gemessen. Die Messfehler betrugen unter 0.1 % des Messbereiches von 150 mm. Ein menschliches Gesicht und eine Schiene wurden ebenfalls untersucht. Die vorgeschlagene Methode ist geeignet für die Formmessung komplexer Objekte. Da nur neun Bilder mit Phasenschritten bei regulären Intervallen benötigt werden, ist die vorgeschlagene Methode auch geeignet für Hochgeschwindigkeits- oder Realzeitmessungen. In der Zukunft wird diese Methode ein leistungsfähiges Werkzeug darstellen für verschiedene Arten der Überprüfung dreidimensionales Profildigitalisieren, oder der Betriebsüberwachung von Transportsystemen oder Betonstrukturen.
    Keywords: Messen Geometrischer Größen ; Form (Gestalt) ; Dreidimensionale Darstellung ; Profilometrie ; Morphometrie ; Optisches Gitter ; CCD-Sensor ; Frequenzmodulation ; Laufzeitverzerrung ; Phasenentzerrung;
    ISSN: 0091-3286
    ISSN: 00361860
    E-ISSN: 15602303
    Source: CrossRef
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