glycogen and Gaucher-Disease

glycogen has been researched along with Gaucher-Disease* in 4 studies

Reviews

2 review(s) available for glycogen and Gaucher-Disease

Article	Year
[Animal models for inborn lysosomal storage diseases (author's transl)]. Jikken dobutsu. Experimental animals, 1977, Volume: 26, Issue:1 Topics: Animals; Cats; Cattle; Disease Models, Animal; Dogs; G(M2) Ganglioside; Gangliosidoses; Gaucher Disease; Glycogen; Glycogen Storage Disease Type II; Glycopeptides; Humans; Leukodystrophy, Globoid Cell; Leukodystrophy, Metachromatic; Lipidoses; Lysosomes; Mannosidases; Metabolism, Inborn Errors; Mice; Niemann-Pick Diseases; Rabbits; Sphingolipids	1977
Degenerative disease of the central nervous system. Advances in pediatrics, 1969, Volume: 16 Topics: Angiokeratoma; Arthritis; Autopsy; Biopsy; Carbohydrate Metabolism, Inborn Errors; Central Nervous System Diseases; Child; Diffuse Cerebral Sclerosis of Schilder; Encephalitis; Encephalomyelitis; Gangliosides; Gaucher Disease; Glycogen; Glycosaminoglycans; Humans; Lipid Metabolism; Lipidoses; Medical History Taking; Metabolic Diseases; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Multiple Sclerosis; Myelin Sheath; Nerve Degeneration; Neurons; Niemann-Pick Diseases; Retinal Degeneration; Slow Virus Diseases; Virus Diseases	1969

Article

Year

[Animal models for inborn lysosomal storage diseases (author's transl)].

Jikken dobutsu. Experimental animals, 1977, Volume: 26, Issue:1

Topics: Animals; Cats; Cattle; Disease Models, Animal; Dogs; G(M2) Ganglioside; Gangliosidoses; Gaucher Disease; Glycogen; Glycogen Storage Disease Type II; Glycopeptides; Humans; Leukodystrophy, Globoid Cell; Leukodystrophy, Metachromatic; Lipidoses; Lysosomes; Mannosidases; Metabolism, Inborn Errors; Mice; Niemann-Pick Diseases; Rabbits; Sphingolipids

1977

Degenerative disease of the central nervous system.

Advances in pediatrics, 1969, Volume: 16

Topics: Angiokeratoma; Arthritis; Autopsy; Biopsy; Carbohydrate Metabolism, Inborn Errors; Central Nervous System Diseases; Child; Diffuse Cerebral Sclerosis of Schilder; Encephalitis; Encephalomyelitis; Gangliosides; Gaucher Disease; Glycogen; Glycosaminoglycans; Humans; Lipid Metabolism; Lipidoses; Medical History Taking; Metabolic Diseases; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Multiple Sclerosis; Myelin Sheath; Nerve Degeneration; Neurons; Niemann-Pick Diseases; Retinal Degeneration; Slow Virus Diseases; Virus Diseases

1969

Other Studies

2 other study(ies) available for glycogen and Gaucher-Disease

Article	Year
In vitro inhibition of glycogen-degrading enzymes and glycosidases by six-membered sugar mimics and their evaluation in cell cultures. Bioorganic & medicinal chemistry, 2008, Aug-01, Volume: 16, Issue:15 We investigated in vitro inhibition of mammalian carbohydrate-degrading enzymes by six-membered sugar mimics and their evaluation in cell cultures. 1-Deoxynojirimycin (DNJ) showed no significant inhibition toward glycogen phosphorylase (GP) but was a potent inhibitor of another glycogen-degrading enzyme, amylo-1,6-glucosidase (1,6-GL), with an IC(50) value of 0.16 microM. In primary rat hepatocytes, the inhibition of glycogen breakdown by DNJ reached plateau at 100 microM with 25% inhibition and then remained unchanged. The potent GP inhibitor 1,4-dideoxy-1,4-imino-D-arabinitol (D-AB1) inhibited hepatic glucose production with an IC(50) value of about 9 microM and the inhibition by D-AB1 was further enhanced in the presence of DNJ. DNJ and alpha-homonojirimycin (HNJ) are very potent inhibitors of rat intestinal maltase, with IC(50) values of 0.13 and 0.08 microM, respectively, and also showed a similar strong inhibition toward maltase in Caco-2 cell model system, with IC(50) value of 0.05 and 0.10 microM, respectively. D-Isofagomine (D-IFG) and L-IFG are competitive and noncompetitive inhibitors of human lysosomal beta-glucosidase (beta-GL), respectively, with K(i) values of 8.4 nM and 6.9 microM. D-IFG increased intracellular beta-GL activity by twofold at 10 microM in Gaucher N370S cell line as an 'active-site-specific' chaperone, and surprisingly a noncompetitive inhibitor L-IFG also increased intracellular beta-GL activity by 1.6-fold at 500 microM. Topics: Animals; Caco-2 Cells; Carbohydrate Conformation; Cells, Cultured; Dose-Response Relationship, Drug; Fibroblasts; Gaucher Disease; Glycogen; Glycoside Hydrolases; Hepatocytes; Humans; Imino Sugars; Lysosomes; Rats; Structure-Activity Relationship	2008
[Peripheral nervous system diseases in morbus Gaucher. New data based on electron microscopy]. Schweizerische medizinische Wochenschrift, 1967, Sep-02, Volume: 97, Issue:35 Topics: Adult; Female; Gaucher Disease; Glycogen; Glycolipids; Histocytochemistry; Humans; Liver; Microscopy, Electron; Neurofibrils; Peripheral Nerves; Peripheral Nervous System Diseases; Schwann Cells	1967

Article

Year

In vitro inhibition of glycogen-degrading enzymes and glycosidases by six-membered sugar mimics and their evaluation in cell cultures.

Bioorganic & medicinal chemistry, 2008, Aug-01, Volume: 16, Issue:15

We investigated in vitro inhibition of mammalian carbohydrate-degrading enzymes by six-membered sugar mimics and their evaluation in cell cultures. 1-Deoxynojirimycin (DNJ) showed no significant inhibition toward glycogen phosphorylase (GP) but was a potent inhibitor of another glycogen-degrading enzyme, amylo-1,6-glucosidase (1,6-GL), with an IC(50) value of 0.16 microM. In primary rat hepatocytes, the inhibition of glycogen breakdown by DNJ reached plateau at 100 microM with 25% inhibition and then remained unchanged. The potent GP inhibitor 1,4-dideoxy-1,4-imino-D-arabinitol (D-AB1) inhibited hepatic glucose production with an IC(50) value of about 9 microM and the inhibition by D-AB1 was further enhanced in the presence of DNJ. DNJ and alpha-homonojirimycin (HNJ) are very potent inhibitors of rat intestinal maltase, with IC(50) values of 0.13 and 0.08 microM, respectively, and also showed a similar strong inhibition toward maltase in Caco-2 cell model system, with IC(50) value of 0.05 and 0.10 microM, respectively. D-Isofagomine (D-IFG) and L-IFG are competitive and noncompetitive inhibitors of human lysosomal beta-glucosidase (beta-GL), respectively, with K(i) values of 8.4 nM and 6.9 microM. D-IFG increased intracellular beta-GL activity by twofold at 10 microM in Gaucher N370S cell line as an 'active-site-specific' chaperone, and surprisingly a noncompetitive inhibitor L-IFG also increased intracellular beta-GL activity by 1.6-fold at 500 microM.

Topics: Animals; Caco-2 Cells; Carbohydrate Conformation; Cells, Cultured; Dose-Response Relationship, Drug; Fibroblasts; Gaucher Disease; Glycogen; Glycoside Hydrolases; Hepatocytes; Humans; Imino Sugars; Lysosomes; Rats; Structure-Activity Relationship

2008

[Peripheral nervous system diseases in morbus Gaucher. New data based on electron microscopy].

Schweizerische medizinische Wochenschrift, 1967, Sep-02, Volume: 97, Issue:35

Topics: Adult; Female; Gaucher Disease; Glycogen; Glycolipids; Histocytochemistry; Humans; Liver; Microscopy, Electron; Neurofibrils; Peripheral Nerves; Peripheral Nervous System Diseases; Schwann Cells

1967