نتایج جستجو برای: bckd deficiency
تعداد نتایج: 137174 فیلتر نتایج به سال:
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,...
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 ...
(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...
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...
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...
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...
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-...
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...
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...
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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