نتایج جستجو برای: aspartoacylase enzyme

تعداد نتایج: 241038  

Journal: :Neuroscience letters 2004
S Surendran E L Ezell M J Quast J Wei S K Tyring K Michals-Matalon R Matalon

Canavan disease (CD) is an autosomal recessive disorder caused by aspartoacylase deficiency leading to accumulation of N-acetylaspartic acid and spongy degeneration of the brain. The mouse model for CD showed low levels of glutamate and gamma-aminobutyric acid (GABA) in the brain. Whether the low levels of glutamate and GABA observed in the CD mouse brain lead to abnormal production of glutamat...

Journal: :Brain research bulletin 2003
Sankar Surendran Peter L Rady Kimberlee Michals-Matalon Michael J Quast David K Rassin Gerald A Campbell Ed L Ezell Jingna Wei Stephen K Tyring Sylvia Szucs Reuben Matalon

Canavan disease (CD) is an autosomal recessive leukodystrophy characterized by spongy degeneration of the brain. The clinical features of CD are hypotonia, megalencephaly, and mental retardation leading to early death. While aspartoacylase (ASPA) activity increases with age in the wild type mouse brain, there is no ASPA activity in the CD mouse brain. So far ASPA deficiency and elevated NAA hav...

Journal: :Molecular therapy : the journal of the American Society of Gene Therapy 2013
Seemin Seher Ahmed Huapeng Li Chunyan Cao Elif M Sikoglu Andrew R Denninger Qin Su Samuel Eaton Ana A Liso Navarro Jun Xie Sylvia Szucs Hongwei Zhang Constance Moore Daniel A Kirschner Thomas N Seyfried Terence R Flotte Reuben Matalon Guangping Gao

Canavan's disease (CD) is a fatal pediatric leukodystrophy caused by mutations in aspartoacylase (AspA) gene. Currently, there is no effective treatment for CD; however, gene therapy is an attractive approach to ameliorate the disease. Here, we studied progressive neuropathology and gene therapy in short-lived (≤ 1 month) AspA(-/-) mice, a bona-fide animal model for the severest form of CD. Sin...

Journal: :acta medica iranica 0
h. vatandoost

pretreatment of adults of iraq strain of afhstephenst with a sub-letlial dose of pcrmethrin resulted in a 65% enliancemenl of tolerance to this insecticide. similarly, pre-exposure of dub-lpr larvae to a sub-lethal dose of pcrmethrin resulted in increased tolerance to pcrmethrin. for the sensitive ind-s strain, the inductive effect was smaller. tiie results of bioassays of resistant and suscept...

Journal: :Molecular genetics and metabolism 2006
B J Zeng Z H Wang P A Torres G M Pastores P Leone S S Raghavan E H Kolodny

Canavan disease (CD), an autosomal recessive neurodegenerative disorder, is caused by mutations in the aspartoacylase (ASPA) gene. In the present study, the ASPA gene was analyzed in 24 non-Jewish patients with CD from 23 unrelated families. Within this cohort, we found three large novel deletions of approximate 92, 56, and 12.13 kb in length, using both self-ligation of restriction endonucleas...

Journal: :The Science of the total environment 2012
Sebastian Beggel Inge Werner Richard E Connon Juergen P Geist

The utilization of molecular endpoints in ecotoxicology can provide rapid and valuable information on immediate organismal responses to chemical stressors and is increasingly used for mechanistic interpretation of effects at higher levels of biological organization. This study contributes knowledge on the sublethal effects of a commonly used insecticide, the phenylpyrazole fipronil, on larval f...

Journal: :nanomedicine journal 0
narges babadaie samani department of biochemistry, falavarjan branch, islamic azad university, isfahan, iranسازمان اصلی تایید شده: دانشگاه آزاد اسلامی علوم و تحقیقات (islamic azad university science and research branch) hashem nayeri department of biochemistry, falavarjan branch, islamic azad university, isfahan, iranسازمان اصلی تایید شده: دانشگاه آزاد اسلامی علوم و تحقیقات (islamic azad university science and research branch) gholamreza amiri amiri falavarjan branch, islamic azad university, isfahan, iranسازمان اصلی تایید شده: دانشگاه آزاد اسلامی علوم و تحقیقات (islamic azad university science and research branch)

objective(s): enzyme immobilization via nanoparticles is perfectly compatible against the other chemical or biological approximate to improve enzyme functions and stability. in this study lactoperoxidase was immobilized onto silica-coated magnetite nanoparticles to improve enzyme properties in the presence of cadmium chloride as an inhibitor. materials and methods:  the process consists of the ...

Journal: :iranian journal of allergy, asthma and immunology 0
talat mokhtari azad anahid ehteda parvin yavari r. hamkar zahra safar pour m. essalat rakhsheh nategh

laboratory diagnosis of acute measles is usually achieved by serology assays for measle-specific igm antibody. for comparison of measle-specific igm antibody in saliva and serum, 95 paired blood and saliva samples were collected 1-14 days after the onset of rash. the specimens were tested for specific igm antibody by an igm antibody-capture enzyme immunoassay (eia). measles igm antibody was det...

Journal: :iranian journal of allergy, asthma and immunology 0
behzad hooshm alireza monsef mohammad amirmadglesi mani kashani

the aim of this study was to evaluate an enzyme - linked immunosorbent assay (elisa) for the detection of anti - helicobacter pylori (h. pylori) specific igg antibodies in specimens of oral fluid. all subjects over the age 18 years undergoing endoscopy for any reason were asked to participate in the study. two groups of 44 patients in each were selected as hp+ and hp-. at the same time, 5 milli...

Journal: :international journal of molecular and clinical microbiology 0
mikhail lakhtin institute: g.n. gabrichevsky research institute for epidemiology and microbiology, russia vladimir lakhtin institute: g.n. gabrichevsky research institute for epidemiology and microbiology, russia vladimir alyoshkin institute: g.n. gabrichevsky research institute for epidemiology and microbiology, russia

examples of inter- and intramolecular direct co-functioning of lectins and enzymes are summarized. the data indicate importance of lectin-enzyme relationships in molecular and clinical microbiology and medical biotechnology. lectin-enzyme relationships have extended prospects in medicine and biotechnology.

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