نتایج جستجو برای: hadh

تعداد نتایج: 56  

Journal: :American journal of human genetics 2013
Sarah E Flanagan Weijia Xie Richard Caswell Annet Damhuis Christine Vianey-Saban Teoman Akcay Feyza Darendeliler Firdevs Bas Ayla Guven Zeynep Siklar Gonul Ocal Merih Berberoglu Nuala Murphy Maureen O'Sullivan Andrew Green Peter E Clayton Indraneel Banerjee Peter T Clayton Khalid Hussain Michael N Weedon Sian Ellard

Next-generation sequencing (NGS) enables analysis of the human genome on a scale previously unachievable by Sanger sequencing. Exome sequencing of the coding regions and conserved splice sites has been very successful in the identification of disease-causing mutations, and targeting of these regions has extended clinical diagnostic testing from analysis of fewer than ten genes per phenotype to ...

Journal: :Acta crystallographica. Section F, Structural biology communications 2014
Dongwen Zhou Wonnop Visessanguan Siriporn Chaikaew Soottawat Benjakul Kohei Oda Alexander Wlodawer

Histamine dehydrogenase (HADH) catalyzes the oxidative deamination of histamine, resulting in the production of imidazole acetaldehyde and an ammonium ion. The enzyme isolated from the newly identified halophilic archaeon Natrinema gari BCC 24369 is significantly different from the previously described protein from Nocardioides simplex. This newly identified HADH comprises three subunits with m...

2017
Owen M Wilkins Alexander J Titus Jiang Gui Melissa Eliot Rondi A Butler Erich M Sturgis Guojun Li Karl T Kelsey Brock C Christensen

Polymorphisms in microRNAs and their target sites can disrupt microRNA-dependent gene regulation, and have been associated with cancer susceptibility. However, genome-scale analyses of microRNA-related genetic variation in cancer are lacking. We tested the associations of ~40 000 common [minor allele frequency (MAF) ≥5%], microRNA-related single nucleotide polymorphisms (miR-SNPs), with risk of...

Journal: :Brain research 2004
Sarah F Leibowitz Jordan T Dourmashkin Guo-Qing Chang James O Hill Ellis C Gayles Susan K Fried Jian Wang

To compare the effects of acute exposure to dietary fat to those of chronic exposure, Sprague-Dawley rats were given a high-fat diet (50% fat) or moderate-fat diet (25% fat) for 1 day, 2 h or 3 weeks. With measurements of various parameters, the high-fat diet for 21 days produced the expected changes of: (1) a significant increase in total caloric intake and dissected fat pad weights; (2) a ris...

2015
Zi‐chuan Fan Jin‐wen Ni Lin Yang Li‐yuan Hu Si‐min Ma Mei Mei Bi‐jun Sun Hui‐jun Wang Wen‐hao Zhou

Congenital hyperinsulinism (CHI) has been mostly associated with mutations in seven major genes. We retrospectively reviewed a cohort of 32 patients with CHI. Extensive mutational analysis (ABCC8,KCNJ11,GCK,GLUD1,HADH,HNF4A, and UCP2) was performed on Ion torrent platform, which could analyze hundreds of genes simultaneously with ultrahigh-multiplex PCR using up to 6144 primer pairs in a single...

Journal: :Thorax 2000
F Maltais P LeBlanc F Whittom C Simard K Marquis M Bélanger M J Breton J Jobin

BACKGROUND Enzymatic and histochemical abnormalities of the peripheral muscle may play a role in exercise intolerance in patients with chronic obstructive pulmonary disease (COPD). A study was undertaken to measure the mitochondrial enzyme activity of the vastus lateralis muscle in patients with COPD and to evaluate the relationship between enzyme activities and functional status. METHODS Fif...

Journal: :international journal of endocrinology and metabolism 0
mohammad reza alaei department of pediatric endocrinology, mofid children’s hospital, shahid beheshti university of medical sciences, tehran, ir iran susan akbaroghli genetic counseling division, mofid children’s hospital, shahid beheshti university of medical sciences, tehran, ir iran; mofid children’s hospital, tehran, ir iran. tel: +98-2122227033, fax: +98-2122227033 mohammad keramatipour department of medical genetics, school of medicine, tehran university of medical sciences, tehran, ir iran ali alaei school of medicine, shahid beheshti university of medical sciences, tehran, ir iran

conclusions congenital hyperinsulinism can have different inheritance pattern. autosomal recessive inheritance is more common but less frequently autosomal dominant inheritance can be seen. it appears that mutations in abcc8 gene can show both autosomal recessive and autosomal dominant inheritance of the disease. pcr followed by sanger sequencing proved to be an efficient method for mutation de...

Journal: :FEBS letters 1981
H M Holden L J Banaszak C Frieden D J McLoughlin

L-3-Hydroxyacyl coenzyme A dehydrogenase (L-3hydroxyacyl-CoA:NAD oxidoreductase; EC 1 .l .1.35) is a mitochondrial enzyme involved in the beta oxidation of fatty acids. The enzyme, as isolated from pig heart muscle, hasM, 67 000 and contains two identical subunits [l]. The amino acid sequence has recently been completed and appears to have little homology with other dehydrogenases [2]. Like man...

2013
Christian Roy Sabina Paglialunga Gert Schaart Esther Moonen-Kornips Ruth C. Meex Esther Phielix Joris Hoeks Matthijs K. C. Hesselink Katherine Cianflone Patrick Schrauwen

OBJECTIVE To investigate the role of Acylation Stimulating Protein (ASP) receptor C5L2 in skeletal muscle fatty acid accumulation and metabolism as well as insulin sensitivity in both mice and human models of diet-induced insulin resistance. DESIGN AND METHODS Male wildtype (WT) and C5L2 knockout (KO) mice were fed a low (LFD) or a high (HFD) fat diet for 10 weeks. Intramyocellular lipid (IMC...

Journal: :Journal of molecular endocrinology 2015
Sofia A Rahman Azizun Nessa Khalid Hussain

Congenital hyperinsulinism (CHI) is a complex heterogeneous condition in which insulin secretion from pancreatic β-cells is unregulated and inappropriate for the level of blood glucose. The inappropriate insulin secretion drives glucose into the insulin-sensitive tissues, such as the muscle, liver and adipose tissue, leading to severe hyperinsulinaemic hypoglycaemia (HH). At a molecular level, ...

نمودار تعداد نتایج جستجو در هر سال

با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید