Continuous glucose for treatment of patients with type 1 glycogen-storage disease: comparison of the effects of dextrose and uncooked cornstarch on biochemical variables

1990 ◽  
Vol 52 (6) ◽  
pp. 1043-1050 ◽  
Author(s):  
J I Wolfsdorf ◽  
R A Plotkin ◽  
L M Laffel ◽  
J F Crigler
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Biochemical Variables ◽  
Uncooked Cornstarch

Bone mineralisation in type 1 glycogen storage disease

1995 ◽  
Vol 154 (6) ◽  
pp. 483-487 ◽  
Author(s):  
Philip J. Lee ◽  
Jatin S. Patel ◽  
Mary Fewtrell ◽  
James V. Leonard ◽  
Nicholas J. Bishop
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Bone Mineralisation

scholarly journals A Molecular Link between the Common Phenotypes of Type 1 Glycogen Storage Disease and HNF1α-null Mice

2000 ◽  
Vol 276 (11) ◽  
pp. 7963-7967 ◽  
Author(s):  
Hisayuki Hiraiwa ◽  
Chi-Jiunn Pan ◽  
Baochuan Lin ◽  
Taro E. Akiyama ◽  
Frank J. Gonzalez ◽  
...  
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage ◽  
The Common

scholarly journals Hypogonadotropic Hypogonadism in Males with Glycogen Storage Disease Type 1

2017 ◽  
pp. 79-84 ◽  
Author(s):  
Evelyn M. Wong ◽  
Anna Lehman ◽  
Philip Acott ◽  
Jane Gillis ◽  
Daniel L. Metzger ◽  
...  
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Hypogonadotropic Hypogonadism ◽  
Glycogen Storage Disease Type ◽  
Glycogen Storage ◽  
Disease Type

scholarly journals Liver Microsomal Transport of Glucose-6-Phosphate, Glucose, and Phosphate in Type 1 Glycogen Storage Diseases1

1998 ◽  
Vol 83 (1) ◽  
pp. 224-229
Author(s):  
P. Marcolongo ◽  
G. Bánhegyi ◽  
A. Benedetti ◽  
C. J. Hinds ◽  
A. Burchell
Keyword(s):  
Light Scattering ◽  
Kinetic Analysis ◽  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Liver Microsomes ◽  
Glycogen Storage ◽  
Microsomal Membrane ◽  
Scattering Method ◽  
Phosphatase System

The transport of glucose-6-phosphate (G6P), glucose, and orthophosphate into liver microsomes, isolated from six patients with various subtypes of type 1 glycogen storage disease (GSD), was measured using a light-scattering method. We found that G6P, glucose, and phosphate could all cross the microsomal membrane, in four cases of type 1a GSD. In contrast, liver microsomal transport of G6P and phosphate was deficient in the GSD 1b and 1c patients, respectively. These results support the involvement of multiple proteins (and genes) in GSD type 1. The results obtained with the light-scattering method are in accordance with conventional kinetic analysis of the microsomal glucose-6-phosphatase system. Therefore, this technique could be used to directly diagnose type 1b and 1c GSD.

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