β-Ureidopropionase deficiency due to novel and rare UPB1 mutations affecting pre-mRNA splicing and protein structural integrity and catalytic activity

Autor: Doreen Dobritzsch, Judith Meijer, Rutger Meinsma, Dirk Maurer, Ardeshir A. Monavari, Anders Gummesson, Annika Reims, Jorge A. Cayuela, Natalia Kuklina, Jean-François Benoist, Laurence Perrin, Birgit Assmann, Georg F. Hoffmann, Jörgen Bierau, Angela M. Kaindl, André B.P. van Kuilenburg

Publikováno v: Molecular genetics and metabolism, 136(3), 177-185. Academic Press Inc.
Molecular Genetics and Metabolism, 136(3), 177-185. Academic Press
Molecular Genetics and Metabolism, 136(3), 177-185. Academic Press Inc.

beta-Ureidopropionase is the third enzyme of the pyrimidine degradation pathway and catalyses the conversion of N-carbamyl-beta-alanine and N-carbamyl-beta-aminoisobutyric acid to beta-alanine and beta-aminoisobutyric acid, ammonia and CO2. To date,

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4548d2ad17ee7613703e5f517be70cb1
https://doi.org/10.1016/j.ymgme.2022.01.102

Comparison of the cubic equations of state and different transport properties models for ORC turbines modeling

Autor: Aleksandr Laskin, Natalia Kuklina, Maksim Smirnov, Aleksandr Sebelev, Kirill Lapshin

Publikováno v: European Conference on Turbomachinery Fluid Dynamics and hermodynamics.

The present paper focuses on a comparison of cubic equations of state (EoS) with accurate Span-Wagner (SW) EoS. Aungier Redlich-Kwong (ARK) EoS, Soave Redlich-Kwong (SRK) EoS and standard Peng-Robinson (PR) EoS were chosen as the basic EoS implemente

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::e72384f102649a6597c1c1cf779ad9cf
https://doi.org/10.29008/etc2019-141

Effects of hub endwall geometry and rotor leading edge shape on performance of supersonic axial impulse turbine. Part I

Autor: Nikolai Zabelin, Natalia Kuklina, Aleksandr Sebelev, Maksim Smirnov

Publikováno v: European Conference on Turbomachinery Fluid Dynamics and hermodynamics.

The paper focuses on methods for an efficiency increasing of supersonic axial impulse turbines. There was chosen the axial turbine stage with axisymmetric nozzles and mean diameter 103.5 mm as the base model for a numerical and experimental investiga

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::049ad2b573321f7c3a5e02677f35a557
https://doi.org/10.29008/etc2017-100