Results 171 to 180 of about 6,311 (212)

Glycogen branching enzyme in Lafora myoclonus epilepsy

Biochemical Medicine, 1982
Abstract Glycogen branching enzymes (EC 2.4.1.18) in extracts of cerebral cortex from a patient with Lafora myoclonus epilepsy and several normal subjects were compared in terms of activity and physical properties of the proteins. Branching-enzyme activity in Lafora cortex tissue was significantly higher than in controls. Gel-exclusion chromatography
C P, Zimmerman, A M, Gold
openaire   +2 more sources

Cloning and characterization of the glycogen branching enzyme gene existing in tandem with the glycogen debranching enzyme from Pectobacterium chrysanthemi PY35

Biochemical and Biophysical Research Communications, 2003
The glycogen branching enzyme gene (glgB) from Pectobacterium chrysanthemi PY35 was cloned, sequenced, and expressed in Escherichia coli. The glgB gene consisted of an open reading frame of 2196bp encoding a protein of 731 amino acids (calculated molecular weight of 83,859Da).
Sang Ryeol Park, Su Young Hong, Han Dae Yun   +2 more
exaly   +3 more sources

The glgB-encoded glycogen branching enzyme is essential for glycogen accumulation in Corynebacterium glutamicum

Microbiology, 2011
Corynebacterium glutamicum transiently accumulates glycogen as carbon capacitor during the early exponential growth phase in media containing carbohydrates. In some bacteria glycogen is synthesized by the consecutive action of ADP-glucose pyrophosphorylase (GlgC), glycogen synthase (GlgA) and glycogen branching enzyme (GlgB).
Gerd M, Seibold, Katrin J, Breitinger, Raoul, Kempkes, Leonard, Both, Matthias, Krämer, Stefan, Dempf, Bernhard J, Eikmanns   +6 more
openaire   +2 more sources

Glycogen Storage Disorder due to Glycogen Branching Enzyme (GBE) Deficiency: A Diagnostic Dilemma

Ultrastructural Pathology, 2015
Glycogen branching enzyme deficiency/Andersen disease can manifest with a spectrum of clinical phenotypes, making the diagnosis difficult. An 11-year-old Pakistani boy presented with a history of progressive weakness and delayed milestones. Echocardiography showed features of dilated cardiomyopathy.
Aanchal Kakkar, Aruna Nambirajan, Chitra Sarkar   +2 more
exaly   +3 more sources

Schistosoma haematobium: Histochemistry of glycogen, glycogen phosphorylase a and glycogen branching enzyme in niridazole-treated females

International Journal for Parasitology, 1992
The body posterior to the ovary of Schistosoma haematobium females was investigated. Glycogen, glycogen phosphorylase a (EC 2.4.1.1) and glycogen branching enzyme (EC 2.4.1.18) activities were detected in the subtegumental muscle system, parenchyma and mature vitelline cells, whereas no activities were detected in the tegument and immature vitelline ...
Moczon T, Swiderski Z
openaire   +3 more sources

Differentiated structure of synthetic glycogen-like particle by the combined action of glycogen branching enzymes and amylosucrase

International Journal of Biological Macromolecules, 2022
Glycogen-like particles (GLPs) were built up from sucrose by applying de novo one-pot enzymatic process of amylosucrase (ASase; 6 U·mL-1) and glycogen branching enzymes (GBEs; 0.001 and 0.005 U·mL-1). Due to different chain-length transferring patterns of GBEs, structurally differentiated GLPs were synthesized.
Daeyeon, Lee, Sang-Dong, Park, Su-Jin, Jun, Jong-Tae, Park, Pahn-Shick, Chang, Sang-Ho, Yoo   +5 more
openaire   +2 more sources

Alteration of mitochondrial function in the livers of mice with glycogen branching enzyme deficiency

Biochimie, 2021
Glycogen storage disease type IV (GSD IV) is caused by mutations in the glycogen branching enzyme gene (GBE1) that lead to the accumulation of aberrant glycogen in affected tissues, mostly in the liver. To determine whether dysfunctional glycogen metabolism in GSD IV affects other components of cellular bioenergetics, we studied mitochondrial function ...
Dominika Malinska, Giorgia Testoni, Malgorzata Bejtka, Jordi Duran, Joan J. Guinovart, Jerzy Duszynski   +5 more
openaire   +2 more sources

Structural features of the glycogen branching enzyme encoding genes from aspergilli

Microbiological Research, 2002
A maltose binding protein, p78, was purified to homogeneity from Aspergillus nidulans by a single column chromatography step on cross-linked amylose. The partial amino acid sequence was highly homologous to the glycogen branching enzymes (GBEs) of human and yeast, and p78 did show branching enzyme activity.
Masashi Kato, Tetsuo Kobayashi, Norihiro Tsukagoshi   +2 more
exaly   +3 more sources

Isolation and characterization of glycogen branching enzyme from rabbit liver

Biochemistry, 1983
Glycogen branching enzyme was isolated from rabbit liver. The highly purified enzyme shows a monomer molecular weight of 71 000 by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and apparent molecular weights of 93 000 by sucrose density gradient sedimentation and 52 000 by gel-exclusion chromatography on Sephacryl S-300.
C P, Zimmerman, A M, Gold
openaire   +2 more sources

Glycogen branching enzyme deficiency in adult polyglucosan body disease

Annals of Neurology, 1993
AbstractBranching enzyme activity was assayed in muscle, peripheral nerve, and leukocytes from 2 Ashkenazi‐Jewish patients with adult polyglucosan body disease and 1 African‐American and 3 Caucasian patients with the same clinical and pathological features.
Bruno C., Servidei S., Shanske S., Karpati G., Carpenter S., McKee D., Barohn R. J., Hirano M., Rifai Z., Dimauro S.   +9 more
openaire   +2 more sources