نتایج جستجو برای: glutaric academia

تعداد نتایج: 18388  

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2006
Tamar Evan-Salem Inbal Baruch Liat Avram Yoram Cohen Liam C Palmer Julius Rebek

The host-guest complexes of resorcin[4]arenes with small molecules in organic solutions are examined using modern NMR spectroscopic methods. The complexation of glutaric acid and beta-methyl d-glucopyranoside in chloroform were investigated through 2D COSY, 2D NOESY, 1D NOE, and diffusion-ordered NMR spectroscopy (DOSY) techniques. These methods indicate that the complex is a self-assembled cap...

Journal: :Brain : a journal of neurology 2010
Kevin A Strauss Patrick Donnelly Max Wintermark

In glutaric aciduria type 1, glutaryl-coenzyme A and its derivatives are produced from intracerebral lysine and entrapped at high concentrations within the brain, where they interfere with energy metabolism. Biochemical toxicity is thought to trigger stroke-like striatal degeneration in susceptible children under 2 years of age. Here, we explore vascular derangements that might also contribute ...

2016
Jae Ho Shin Seok Hyun Park Young Hoon Oh Jae Woong Choi Moon Hee Lee Jae Sung Cho Ki Jun Jeong Jeong Chan Joo James Yu Si Jae Park Sang Yup Lee

BACKGROUND 5-Aminovaleric acid (5AVA) is an important five-carbon platform chemical that can be used for the synthesis of polymers and other chemicals of industrial interest. Enzymatic conversion of L-lysine to 5AVA has been achieved by employing lysine 2-monooxygenase encoded by the davB gene and 5-aminovaleramidase encoded by the davA gene. Additionally, a recombinant Escherichia coli strain ...

Journal: :iranian journal of child neurology 0
seyyed hassan tonekaboni associate professor of pediatric neurology, pediatric neurology research center, shahid beheshti university of medical sciences(sbmu), tehran, iran

inborn errors of organic acid metabolism are relatively recently recognized diseases with a wide spectrum of clinical signs and symptoms: ranging from asymptomatic, normal appearing children to death during first few days of life.in my presentation i will try to explain some of the most common clinical presentation of these disorder with stress on neurologic findings. organic acidemia usually h...

Journal: :Angewandte Chemie 2013
Ranjit Thakuria Mark D Eddleston Ernest H H Chow Gareth O Lloyd Barry J Aldous Joseph F Krzyzaniak Andrew D Bond William Jones

AFM of cocrystals: Atomic force microscopy can be used to observe phase changes at crystal surfaces where the transformation is accompanied by a change in the spacing between layers of molecules. The conversion of a metastable polymorph of the caffeine-glutaric acid cocrystal into the thermodynamically stable form was analyzed continuously in situ using intermittent-contact-mode atomic force mi...

Journal: :Human molecular genetics 2002
David M Koeller Michael Woontner Linda S Crnic Bette Kleinschmidt-DeMasters Janet Stephens Edgar L Hunt Stephen I Goodman

Glutaric acidemia type I (GA-I) is an autosomal recessive disorder of amino acid metabolism resulting from a deficiency of glutaryl-CoA dehydrogenase (GCDH). Patients accumulate glutaric acid (GA) and 3-OH glutaric acid (3-OHGA) in their blood, urine and CSF. Clinically, GA-I is characterized by macrocephaly, progressive dystonia and dyskinesia. Degeneration of the caudate and putamen of the ba...

2008
A. A. Zardini S. Sjogren C. Marcolli U. K. Krieger M. Gysel E. Weingartner U. Baltensperger

Atmospheric aerosols are often mixtures of inorganic and organic material. Organics can represent a large fraction of the total aerosol mass and are comprised of water-soluble and insoluble compounds. Increasing attention was paid in the last decade to the capability of mixed inorganic/organic aerosol particles to take up water (hygroscopicity). We performed hygroscopicity measurements of inter...

Journal: :Journal of Inherited Metabolic Disease 1999

Journal: :Developmental Medicine & Child Neurology 2009

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