نتایج جستجو برای: bckd deficiency

تعداد نتایج: 137174  

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2013
Shih-Chia Tso Xiangbing Qi Wen-Jun Gui Jacinta L Chuang Lorraine K Morlock Amy L Wallace Kamran Ahmed Sunil Laxman Philippe M Campeau Brendan H Lee Susan M Hutson Benjamin P Tu Noelle S Williams Uttam K Tambar R Max Wynn David T Chuang

The branched-chain amino acids (BCAAs) leucine, isoleucine, and valine are elevated in maple syrup urine disease, heart failure, obesity, and type 2 diabetes. BCAA homeostasis is controlled by the mitochondrial branched-chain α-ketoacid dehydrogenase complex (BCKDC), which is negatively regulated by the specific BCKD kinase (BDK). Here, we used structure-based design to develop a BDK inhibitor,...

Journal: :iranian journal of allergy, asthma and immunology 0
abolhassan farhoudi zahra chavoshzadeh bahram mir saeid ghazi asghar aghamohammadi mohammad gharagozlou

cd8 deficiency is a rare primary immunodeficiency with low or absent peripheral cd8 cells which results from tap deficiency, zap 70 deficiency and cd8 α gene mutation. we report a 14 year old female who presented with a history of recurrent pneumonia, bronchiectasis, otitis, severe varicella, herpetic lesions of mouth, bilateral uveitis, and cataract formation since the age of 8 years. she had ...

پایان نامه :وزارت علوم، تحقیقات و فناوری - دانشگاه شهید چمران اهواز - دانشکده علوم 1394

(mitochondrial trifunctional protein (mtp که در غشای درونی میتوکندی متصل است،سه مرحله نهایی چرخه بتا اکسیداسیون اسید های چرب را کاتالیز میکند.این کمپلکس یک هترو-اکتامر کمپلکس است که از 8 قسمت(زیرواحد) تشکیل شده است. 4زیرواحد ? شامل (lceh(long-chain2,3-enoyl-coahydratase و (lchad (long-chain 3- hydroxyacyl coa dehydrogenase می باشد و 4 زیرواحد ? که در برگیرنده فعالیت (lckt(long chain 3-ketoacyl...

Journal: :international journal of agricultural management and development 2011
m. govindaraj p. kannan p. arunachalam

the green revolution fulfilled the food demand of crowded millions. from the time of green revolution to till date high yielding and fertilizer responsive varieties have evolved to increase the production per unit area. to improve the productivity only major nutrients are concentrated almost in all crops. though the importance of micronutrient realized during past decades in most of the crops b...

Journal: :iranian journal of allergy, asthma and immunology 0
nima rezaei research center for immunodeficiencies, children's medical center, tehran university of medical sciences, tehran, iran and molecular immunology research center, department of immunology, school of medicine, tehran university of medical sciences, tehran, iran hassan abolhassani research center for immunodeficiencies, children's medical center, tehran university of medical sciences, tehran, iran amir kasraian research center for immunodeficiencies, children's medical center, tehran university of medical sciences, tehran, iran payam mohammadinejad research center for immunodeficiencies, children's medical center, tehran university of medical sciences, tehran, iran bamdad sadeghi research center for immunodeficiencies, children's medical center, tehran university of medical sciences, tehran, iran asghar aghamohammadi research center for immunodeficiencies, children's medical center, tehran university of medical sciences, tehran, iran

common variable immunodeficiency (cvid) and selective iga deficiency (sigad) are the most common primary antibody deficiencies. these two diseases may have coincidence in one family and sigad can progress to cvid which suggest common underlying genetic defects between sigad and cvid. this study was designed to find the prevalence of multiple cases in families of iranian patients with cvid or si...

Journal: :iranian journal of science and technology (sciences) 2012
s. mohsenzadeh

fennel (foeniculum vulgare mill.) is widely cultivated for its edible, strongly-flavored leaves, fruits, seeds andmedicinal uses. as the wild and cultivated plants are influenced by environmental stresses, the effects of salt (25, 50 and 75 mm nacl), nitrogen and iron deficiency (0 n, 0.5 n of hoagland’s solution, 0 fe and 0.5 fe ofhoagland’s solution), 2°c cold (2, 3 and 4 h) and drought (3, 5...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2001
M Machius J L Chuang R M Wynn D R Tomchick D T Chuang

Mitochondrial protein kinases (mPKs) are molecular switches that down-regulate the oxidation of branched-chain alpha-ketoacids and pyruvate. Elevated levels of these metabolites are implicated in disease states such as insulin-resistant Type II diabetes, branched-chain ketoaciduria, and primary lactic acidosis. We report a three-dimensional structure of a member of the mPK family, rat branched-...

2016
Luca A Lotta Robert A Scott Stephen J Sharp Stephen Burgess Jian'an Luan Therese Tillin Amand F Schmidt Fumiaki Imamura Isobel D Stewart John R B Perry Luke Marney Albert Koulman Edward D Karoly Nita G Forouhi Rasmus J O Sjögren Erik Näslund Juleen R Zierath Anna Krook David B Savage Julian L Griffin Nishi Chaturvedi Aroon D Hingorani Kay-Tee Khaw Inês Barroso Mark I McCarthy Stephen O'Rahilly Nicholas J Wareham Claudia Langenberg

BACKGROUND Higher circulating levels of the branched-chain amino acids (BCAAs; i.e., isoleucine, leucine, and valine) are strongly associated with higher type 2 diabetes risk, but it is not known whether this association is causal. We undertook large-scale human genetic analyses to address this question. METHODS AND FINDINGS Genome-wide studies of BCAA levels in 16,596 individuals revealed fi...

Journal: :bulletin of the iranian mathematical society 2013
j. wu z. wu

in this paper, some results of singh, gopalakrishna and kulkarni (1970s) have been extended to higher order derivatives. it has been shown that, if $sumlimits_{a}theta(a, f)=2$ holds for a meromorphic function $f(z)$ of finite order, then for any positive integer $k,$ $t(r, f)sim t(r, f^{(k)}), rrightarrowinfty$ if $theta(infty, f)=1$ and $t(r, f)sim (k+1)t(r, f^{(k)}), rrightarrowinfty$ if $th...

Journal: :journal of research in medical sciences 0
mohammad hassan moaddab isfahan endocrine & metabolism research center ammar hassanzadeh keshteli medical students' research center, school of medicine, isfahan university of medical sciences, isfahan, iran. mansour siavash dastjerdi isfahan endocrine and metabolism research center, isfahan university of medical sciences, isfahan, iran. hasan rezvanian isfahan endocrine and metabolism research center, isfahan university of medical sciences, isfahan, iran. ashraf aminorroaya aminorroaya isfahan endocrine and metabolism research center, isfahan university of medical sciences, isfahan, iran. masoud amini isfahan endocrine and metabolism research center, isfahan university of medical sciences, isfahan, iran.

background : iodine deficiency produces the spectrum of iodine deficiency disorders (idds) including endemic goiter, hypothyroidism, cretinism and congenital anomalies. other factors, including goitrogens and micronutrient deficiencies may influence the prevalence and severity of idds and response to iodine supplementation. an ssociation between zinc and goiter has previously been reported. met...

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