11 research outputs found

    Proteomic profiling of quality protein Maize kernels using mass spectrometry

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    Maize (Zea mays L.) is the third most crucial crop worldwide and is of paramount importance in both humansand livestock diets. Conventional maize varieties have less than half of the amino acids recommended for humannutrition, and this deficiency results in an imbalance of amino acids and low protein content, which has been associatedwith several pathologies, including malnutrition. Thus, different countries have focused on research onfortified foods, such as quality protein maize (QPM) noting that these improved varieties may contain up to 100%more essential amino acids residues than conventional maize. Hence, this study aimed to characterize throughtandem mass spectrometry and bioinformatics analysis, relative expression of polypeptides contained in a hybridvariety of QPM, which allow to identify potential markers with implications in the management and improvementof this crops maintaining their intrinsic characteristics. We identify 262 polypeptides, highlighting those related tomolecular function (catalytic activity, structural molecule activity, and binding) and biological process (cellular andmetabolic process). These results provide the necessary information, not only for the characterization of the QPMproteome through novel tools such as proteomics, but also to describe mechanisms related to different biologicalprocesses such as the embryogenesis, development and growth of grains and eventually plants. PotentiallyIt promotes the discovery of molecular markers (biomarkers) that would allow the improvement of agronomicalprocesses

    Prognostic factors for the late onset Pompe disease with enzyme replacement therapy: From our experience of 4 cases including an autopsy case

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    We report 4 cases of late onset glycogen storage disease type II (GSD II) or Pompe disease (OMIM #232300), under. enzyme replacement therapy (ERT) with recombinant human acid alpha glucosidase (rh-GM, OMIM *606800). in these 4 cases, we focused on the case of a 28-years-old man, whose condition at the ERT starting was the worst and resulted in poor prognosis. the autopsy was done under his family's permission, and revealed severe accumulation of glycogen in his muscle, especially diaphragm or iliopsoas, and pulmonary veno-occlusive disease (PVOD) which resulted in severe pulmonary hypertension (PH). This is the first report of PVOD as the cause of PH in Pompe disease. We studied this case comparing to another 3 cases of late onset Pompe disease under the same course of ERT in our hospital, and the average data of the group of late onset Pompe disease with severe pulmonary insufficiency receiving ERT, supposed that low score of the body mass index (BMI) on the baseline, the presence of specific genotype (p.R600C), and signs of pulmonary dysfunction suggesting PH (tachypnea, ultrasound cardiography data) were factors that influenced the prognosis. for a better prognosis in the late onset Pompe disease, an early diagnosis for the early start of ERT before the onset of respiratory failure should be important, and the deliberate management and care should be needed even after the ERT start, especially for severe cases including pulmonary dysfunction. (C) 2010 Elsevier Inc. All rights reserved.Ministry of Health, Labour and Welfare in JapanJikei Univ, Dept Gene Therapy, Inst DNA Med,Sch Med, Minato Ku, Tokyo, JapanJikei Univ, Sch Med, Dept Pediat, Minato Ku, Tokyo, JapanJikei Univ, Sch Med, Dept Genet Dis & Genome Sci, Minato Ku, Tokyo, JapanTokyo Womens Med Univ, Dept Pediat, Shinjuku Ku, Tokyo, JapanUniversidade Federal de São Paulo, Ctr Referencia Erros Inatos Metab, São Paulo, BrazilUniversidade Federal de São Paulo, Ctr Referencia Erros Inatos Metab, São Paulo, BrazilWeb of Scienc
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