TY - JOUR A1 - Xie, J. A1 - Techritz, S. A1 - Haebel, Sophie A1 - Horn, A. A1 - Neitzel, H. A1 - Klose, J. A1 - Schuelke, M. T1 - A two-dimensional electrophoretic map of human mitochondrial proteins from immortalized lymphoblastoid cell lines: a prerequisite to study mitochondrial disorders in patients N2 - Mitochondrial diseases may be caused by numerous mutations that alter proteins of the respiratory chain and of other metabolic pathways in the mitochondrium. For clinicians this disease group poses a considerable diagnostic challenge due to ambiguous genotype-phenotype relationships. Until now, only 30 % of the mitochondriopathies can be diagnosed at the molecular level. We therefore need a new diagnostic tool that offers a wide view on the mitochondrial proteins. Here, we present a method to generate a high-resolution, large-gel two-dimensional gel electrophoretic (2-DE) map of a purified fraction of mitochondrial proteins from Epstein-Barr virus-immortalized lymphoblastoid cell line (LCL). LCLs can be easily obtained from patients and control subjects in a routine clinical setting. They often express the biochemical phenotype and can be cultured to high cell numbers, sufficient to gain enough purified material for 2- DE. In total we identified 166 mitochondrial proteins. Thirteen proteins were earlier not known to be of mitochondrial origin. Thirty-nine proteins were associated with human diseases ranging from respiratory chain enzyme deficiencies to disorders of P-oxidation and amino acid metabolism. This 2-DE map is intended to be the first step to diagnose mitochondrial diseases at the proteomic level Y1 - 2005 SN - 1615-9853 ER - TY - JOUR A1 - Kehr, Julia A1 - Haebel, Sophie A1 - Blechschmidt-Schneider, Sabine A1 - Willmitzer, Lothar A1 - Steup, Martin A1 - Fisahn, Joachim T1 - Analysis of phloem protein patterns from different organs of Cucurbita maxima Duch. by matrix-assisted laser desorption/ionization time of flight mass spectroscopy combined with sodium dodecyl sufate-polyacryilamide gel electrophoresis Y1 - 1999 ER - TY - JOUR A1 - Rawel, Harshadrai Manilal A1 - Kroll, Jürgen A1 - Riese-Schneider, Brigitte A1 - Haebel, Sophie T1 - Changes in physico-chemical and enzymatic properties of benzyl isothiocyanate derivatized proteinases Y1 - 1998 ER - TY - JOUR A1 - Bahrke, Sven A1 - Einarsson, Jon M. A1 - Gislason, Johannes A1 - Haebel, Sophie A1 - Peter-Katalinic, Jasna T1 - Characterization of chitooligosaccharides by mass spectrometry N2 - Heterochitooligosaccharides of DP 6, DP 9, and DP 12 were evaluated using established methods of derivatization and matrix-assisted laser desorption ionization post source decay mass spectrometry. Y1 - 2003 SN - 82-471-5901-5 ER - TY - THES A1 - Bahrke, Sven A1 - Einarsson, Jon M. A1 - Gislason, Johannes A1 - Haebel, Sophie A1 - Peter-Katalinic, Jasna A1 - Peter, Martin G. T1 - Characterization of chitooligosaccharides by mass spectrometry Y1 - 2003 SN - 82-47-15901-5 ER - TY - JOUR A1 - Ritte, Gerhard A1 - Eckermann, Nora A1 - Haebel, Sophie A1 - Lorberth, Ruth A1 - Steup, Martin T1 - Compartmentation of the starch-related R1 protein in higher plants Y1 - 2000 ER - TY - JOUR A1 - Haebel, Sophie A1 - Albrecht, Tanja A1 - Sparbier, Katrin A1 - Walden, Peter A1 - Körner, Roman A1 - Steup, Martin T1 - Electrophoresis-related protein modification: alkylation of carboxy residues revealed by mass spectrometry Y1 - 1998 ER - TY - JOUR A1 - Tsukamoto, Junko A1 - Haebel, Sophie A1 - Valenca, Gustavo P. A1 - Peter, Martin G. A1 - FRanco, Telma T. T1 - Enzymatic direct synthesis of acrylic acid esters of mono- and disaccharides N2 - Background: There is an increased need to replace materials derived from fossil sources by renewables. Sugar- cane derived carbohydrates are very abundant in Brazil and are the cheapest sugars available in the market, with more than 400 million tons of sugarcane processed in the year 2007. The objective of this work was to study the prepn. of sugar acrylates from free sugars and free acrylic acid, thus avoiding the previous prepn. of protected sugar derivs., such as glycosides, or activated acrylates, such as vinyl acrylate. Results: Lipase catalyzed esterification of three mono- and two disaccharides with acrylic acid, in the presence or absence of mol. sieves was investigated. The reactions were monitored by high-performance liq. chromatog. (HPLC) and the products were analyzed by matrix-assisted laser desorption ionization-time of flight (MALDI-TOF) mass spectrometry. The main products are mono- and diacrylates, while higher esters are formed as minor products. The highest conversion to sugar acrylates was obsd. for the D-glucose and D- fructose, followed by D-xylose and D-maltose. Mol. sieves had no pronounced effect on the conversion. Conclusions: A feasible method is described to produce and to characterize sugar acrylates, including those contg. more than two acrylate groups. The process for prodn. of these higher esters could potentially be optimized further to produce mols. for crosslinking in acrylate polymn. and other applications. The direct enzymic esterification of free carbohydrates with acrylic acid is unprecedented. [on SciFinder (R)]. Y1 - 2008 UR - http://www3.interscience.wiley.com/journal/117946200/grouphome/home.html SN - 0268-2575 ER - TY - JOUR A1 - Asgeirsson, Bjarni A1 - Haebel, Sophie A1 - Thorsteinsson, Leifur A1 - Helgason, Erlendur A1 - Gudmundsson, Kristbjörn O. A1 - Gudmundsson, Gunnar A1 - Roepstorff, Peter T1 - Hereditary cystatin C amyloid angiopathy: monitoring the presence of the Leu-68 -> Gln cystatin C variant in cerebrospinal fluids and monocyte cultures by MS Y1 - 1998 ER - TY - JOUR A1 - Stöckigt, Detlef A1 - Haebel, Sophie T1 - Identification of CID fragments from a protonated glutathione conjugate by isotope-specific MS3 experiments in an ion trap Y1 - 1998 ER -