deoxyfuconojirimycin has been researched along with Glycogen Storage Disease Type II in 1 studies
*Glycogen Storage Disease Type II: An autosomal recessively inherited glycogen storage disease caused by GLUCAN 1,4-ALPHA-GLUCOSIDASE deficiency. Large amounts of GLYCOGEN accumulate in the LYSOSOMES of skeletal muscle (MUSCLE, SKELETAL); HEART; LIVER; SPINAL CORD; and BRAIN. Three forms have been described: infantile, childhood, and adult. The infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy (CARDIOMYOPATHY, HYPERTROPHIC). The childhood form usually presents in the second year of life with proximal weakness and respiratory symptoms. The adult form consists of a slowly progressive proximal myopathy. (From Muscle Nerve 1995;3:S61-9; Menkes, Textbook of Child Neurology, 5th ed, pp73-4) [MeSH]
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 1 (100.00) | 2.80 |
| Authors | Studies |
|---|---|
| Fleet, GWJ; Jia, YM; Kanekiyo, U; Kato, A; Kishida, M; Li, YX; Lu, TT; Nakagome, I; Nash, RJ; Shinzawa, K; Tanaka, N; Yoshida, T; Yoshimura, K; Yu, CY | 1 |
1 other study(ies) available for deoxyfuconojirimycin and Glycogen Storage Disease Type II
| Article | Year |
|---|---|
5-
Topics: 1-Deoxynojirimycin; Alkylation; alpha-Glucosidases; Enzyme Inhibitors; Fibroblasts; Glycogen Storage Disease Type II; Humans; Molecular Dynamics Simulation; Molecular Structure; Mutation; Protein Conformation; Protein Stability; Recombinant Proteins | 2022 |