نتایج جستجو برای: msud

تعداد نتایج: 148  

Journal: :iranian journal of child neurology 0
alireza khatami associate professor of radiology, shahid beheshti university of medical sciences,tehran,iran

organic academia are diverse group of disorders characterized by abnormal degredation of specific amino acid in the specific catabolism due to an enzymeactivity defect which mainly due to enzyme deficiency. the majority of the classic organic acid disorders are caused by abnormal catabolism of branched-chain amino acids like lysine. maple syrup urine disease (msud), propionic acidemia, methylma...

Journal: :PLoS Genetics 2009
Satoshi H. Namekawa Jeannie T. Lee

The sex chromosomes are among the most rapidly evolving and most diverse genetic systems in all of biology. Students of model organisms may, however, have the false impression that there is only one chromosomal mechanism of specifying sex. Among the best-studied metazoans, the XY system is indeed the rule, with inheritance of two X’s determining the female sex (XX), and inheritance of an X and ...

Journal: :iranian journal of child neurology 0
hedieh saneifard assistant professor of pediatric endocrinology shahid beheshti university of medical sciences,tehran,iran

organic acidemias are the group of metabolic disorders which define by high anion gap metabolic acidosis, hypo or hyperglycemia & hyperammonemia.because of the severity of disease in children and its fatality in severe form of disease and also need for life long treatment, prenatal diagnosis is an important diagnostic tool.three approaches to prenatal diagnosis may be possible, including measur...

Journal: : 2022

The article describes the contribution of famous Ukrainian scientist Vasyl Matviyovych Chernyaev to science botany and development herbarium. Biographical data are given.
 Among funds Herbarium (UM) students Uman School Agriculture Horticulture (the successor is National University Horticulture) a valuable historical collection exicates Florae Rossicae (1897–1907). It was established that ...

2014
Rui Chen Nan Jiang Qiyan Jiang Xianjun Sun Yong Wang Hui Zhang Zheng Hu

RNA silencing such as quelling and meiotic silencing by unpaired DNA (MSUD) and several other classes of special small RNAs have been discovered in filamentous fungi recently. More than four different mechanisms of microRNA-like RNAs (milRNAs) production have been illustrated in the model fungus Neurospora crassa including a dicer-independent pathway. To date, very little work focusing on small...

Journal: :Brain 2009
William J. Zinnanti Jelena Lazovic Kathleen Griffin Kristen J. Skvorak Harbhajan S. Paul Gregg E. Homanics Maria C. Bewley Keith C. Cheng Kathryn F. LaNoue John M. Flanagan

Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with life-threatening cerebral oedema and dysmyelination in affected individuals. Treatment requires life-long dietary restriction and monitoring of branched-chain amino acids to avoid brain injury. Despite careful management, children commonly suffer metabolic decompensation in the cont...

2015
Yang Hu

Heterobasidion annosum sensu lato (s.l.) species are destructive pathogens causing root and butt rot in conifers. The species complex consists of five species: H. annosum sensu stricto (s.s.), H. abietinum, H. parviporum, H. irregulare and H. occidentale. The aim of this thesis was to improve the understanding of fungal virulence in this species complex. The comparison of H. irregulare and H. o...

Abbas Sahebghadam Lotfi, Eskander Omidinia, Hamid Shahbaz Mohammadi, Reza Saghiri,

Amino acid dehydrogenases (L-amino acid: oxidoreductase deaminating EC 1.4.1.X) are members of the wider superfamily of oxidoreductases that catalyze the reversible oxidative deamination of an amino acid to its keto acid and ammonia with the concomitant reduction of either NAD+, NADP+ or FAD. These enzymes have been received much attention as biocatalysts for use in biosensors or diagnostic kit...

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