نتایج جستجو برای: sandhoff disease

تعداد نتایج: 1490121  

Journal: :Biochimica et biophysica acta 2013
Ji Woong Choi Jerold Chun

Lysophosphatidic acid (LPA) and sphingosine 1-phosphate (S1P), two of the best-studied lysophospholipids, are known to influence diverse biological events, including organismal development as well as function and pathogenesis within multiple organ systems. These functional roles are due to a family of at least 11 G protein-coupled receptors (GPCRs), named LPA(1-6) and S1P(1-5), which are widely...

Journal: :medical journal of islamic republic of iran 0
ja khan from the department of biochemistry, medical biology center, queen s university of belfast, bt97bl, northern ireland, united kingdom mhr lewis

n-acetyl-b-d-glucosaminidase was purified by affinity and ionexchange chromatography. two major, a and b, and three minor intermediate forms were isolated and characterized. nag-a and nag-b were purified 440 and 1200 fold with final yields of 16 and 23 percent respectively. each activity was represented by a single protein band. after 70 min preincubation at 55°c a loss of70% activity of nag-a ...

2010
Volkan Seyrantepe Pablo Lema Aurore Caqueret Larbi Dridi Samar Bel Hadj Stephane Carpentier Francine Boucher Thierry Levade Lionel Carmant Roy A. Gravel Edith Hamel Pascal Vachon Graziella Di Cristo Jacques L. Michaud Carlos R. Morales Alexey V. Pshezhetsky

Tay-Sachs disease is a severe lysosomal disorder caused by mutations in the HexA gene coding for the α-subunit of lysosomal β-hexosaminidase A, which converts G(M2) to G(M3) ganglioside. Hexa(-/-) mice, depleted of β-hexosaminidase A, remain asymptomatic to 1 year of age, because they catabolise G(M2) ganglioside via a lysosomal sialidase into glycolipid G(A2), which is further processed by β-h...

Journal: :JAMA 2006
Katherine L Wisner Christina Chambers Dorothy K Y Sit

2006;44:421-428. 7. Roumie CL, Elasy TA, Greevy R, et al. Improving blood pressure control through provider education, provider alerts, and patient education. Ann Intern Med. 2006; 145:165-175. 8. Haynes RB, McDonald HP, Garg AX. Helping patients follow prescribed treatment. JAMA. 2002;288:2880-2883. 9. Petrilla AA, Benner JS, Battleman DS, Tierce JC, Hazard EH. Evidence-based interventions to ...

Journal: :Human molecular genetics 2004
Marianne Hammarsund Mikael Lerner Chaoyong Zhu Mats Merup Monika Jansson Gösta Gahrton Hanneke Kluin-Nelemans Stefan Einhorn Dan Grandér Olle Sangfelt Martin Corcoran

Karyotypical alteration of chromosome 5 and in particular band 5q13 is a frequent finding in hairy cell leukemia (HCL). We have previously identified a number of candidate genes localized in close proximity to a constitutional inv(5)(p13.1q13.3) breakpoint in one HCL patient. These included beta-hexosaminodase HEXB, frequently mutated in the lysosomal storage disorder Sandhoff disease. We now r...

2004
Marianne Hammarsund Mikael Lerner Chaoyong Zhu Mats Merup Monika Jansson Gösta Gahrton Hanneke Kluin-Nelemans Stefan Einhorn Dan Grandér Olle Sangfelt Martin Corcoran

Karyotypical alteration of chromosome 5 and in particular band 5q13 is a frequent finding in hairy cell leukemia (HCL). We have previously identified a number of candidate genes localized in close proximity to a constitutional inv(5)(p13.1q13.3) breakpoint in one HCL patient. These included beta-hexosaminodase HEXB, frequently mutated in the lysosomal storage disorder Sandhoff disease. We now r...

پایان نامه :دانشگاه آزاد اسلامی - دانشگاه آزاد اسلامی واحد تهران (پزشکی) - دانشکده پزشکی 1388

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