scholarly journals Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase

2013 ◽  
Vol 127 (1) ◽  
pp. 101-113 ◽  
Author(s):  
Or Kakhlon ◽  
Hava Glickstein ◽  
Naomi Feinstein ◽  
Yan Liu ◽  
Otto Baba ◽  
...  
Keyword(s):  
Glycogen Synthase ◽  
Enzyme Deficiency ◽  
Branching Enzyme ◽  
Glycogen Branching Enzyme

Glycogen branching enzyme deficiency in adult polyglucosan body disease

1993 ◽  
Vol 33 (1) ◽  
pp. 88-93 ◽  
Author(s):  
Claudio Bruno ◽  
Serenella Servidei ◽  
Sara Shanske ◽  
George Karpati ◽  
Stirling Carpenter ◽  
...  
Keyword(s):  
Enzyme Deficiency ◽  
Branching Enzyme ◽  
Polyglucosan Body ◽  
Adult Polyglucosan Body Disease ◽  
Glycogen Branching Enzyme

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

Microbiology ◽  
2011 ◽  
Vol 157 (11) ◽  
pp. 3243-3251 ◽  
Author(s):  
Gerd M. Seibold ◽  
Katrin J. Breitinger ◽  
Raoul Kempkes ◽  
Leonard Both ◽  
Matthias Krämer ◽  
...  
Keyword(s):  
Corynebacterium Glutamicum ◽  
Gene Product ◽  
Glycogen Synthase ◽  
Exponential Growth Phase ◽  
Branching Enzyme ◽  
Glycogen Accumulation ◽  
Glycosidic Bonds ◽  
Its Gene ◽  
Glycogen Branching Enzyme

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). GlgC and GlgA of C. glutamicum have been shown to be necessary for glycogen accumulation in this organism. However, although cg1381 has been annotated as the putative C. glutamicum glgB gene, cg1381 and its gene product have not been characterized and their role in transient glycogen accumulation has not yet been investigated. We show here that the cg1381 gene product of C. glutamicum catalyses the formation of α-1,6-glycosidic bonds in polysaccharides and thus represents a glycogen branching enzyme. RT-PCR experiments revealed glgB to be co-transcribed with glgE, probably encoding a maltosyltransferase. Promoter activity assays with the glgE promoter region revealed carbon-source-dependent expression of the glgEB operon. Characterization of the growth and glycogen content of glgB-deficient and glgB-overexpressing strains showed that the glycogen branching enzyme GlgB is essential for glycogen formation in C. glutamicum. Taken together these results suggest that an interplay of the enzymes GlgC, GlgA and GlgB is not essential for growth, but is required for synthesis of the transient carbon capacitor glycogen in C. glutamicum.

Hallmarks of oxidative stress in the livers of aged mice with mild glycogen branching enzyme deficiency

2020 ◽  
Vol 695 ◽  
pp. 108626
Author(s):  
Dominika Malinska ◽  
Giorgia Testoni ◽  
Jordi Duran ◽  
Alicja Brudnicka ◽  
Joan J. Guinovart ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Enzyme Deficiency ◽  
Branching Enzyme ◽  
Aged Mice ◽  
Glycogen Branching Enzyme

Glycogen branching enzyme deficiency in an infant with severe congenital hypotonia: an emerging diagnosis of muscle weakness in the perinatal period

2009 ◽  
Vol 54 (6) ◽  
pp. 765-768 ◽  
Author(s):  
Cecilia Jimenez-Mallebrera ◽  
Andres Nascimento ◽  
Victoria Cusi ◽  
Joan Ramon Corbera ◽  
Marie-Odile Rolland ◽  
...  
Keyword(s):  
Muscle Weakness ◽  
Perinatal Period ◽  
Enzyme Deficiency ◽  
Branching Enzyme ◽  
Glycogen Branching Enzyme

Muscular weakness and recumbency in a Quarter Horse colt due to glycogen branching enzyme deficiency

2010 ◽  
Vol 15 (4) ◽  
pp. 182-188 ◽  
Author(s):  
B. T. Sponseller ◽  
S. J. Valberg ◽  
T. L. Ward ◽  
A. J. Fales-Williams ◽  
J. R. Mickelson
Keyword(s):  
Enzyme Deficiency ◽  
Muscular Weakness ◽  
Branching Enzyme ◽  
Quarter Horse ◽  
Glycogen Branching Enzyme

Glycogen Branching Enzyme Deficiency

2009 ◽  
pp. 727-728
Author(s):  
David J. Timson ◽  
Richard J. Reece ◽  
James B. Thoden ◽  
Hazel M. Holden ◽  
Andrea L. Utz ◽  
...  
Keyword(s):  
Enzyme Deficiency ◽  
Branching Enzyme ◽  
Glycogen Branching Enzyme
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