butyric acid has been researched along with Glycogen Storage Disease Type II in 1 studies
Butyric Acid: A four carbon acid, CH3CH2CH2COOH, with an unpleasant odor that occurs in butter and animal fat as the glycerol ester.
butyrate : A short-chain fatty acid anion that is the conjugate base of butyric acid, obtained by deprotonation of the carboxy group.
butyric acid : A straight-chain saturated fatty acid that is butane in which one of the terminal methyl groups has been oxidised to a carboxy group.
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)
| Excerpt | Relevance | Reference |
|---|---|---|
| "In fibroblast cells, from adult-onset GSD II patients, D-glucose increased the residual level of alpha-glucosidase activity, suggesting that a structural analogue of d-glucose may be used for enzyme enhancement therapy." | 1.33 | Stabilising normal and mis-sense variant alpha-glucosidase. ( Brooks, DA; Hopwood, JJ; Kakavanos, R; Lang, D; Meikle, PJ, 2006) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (100.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Kakavanos, R | 1 |
| Hopwood, JJ | 1 |
| Lang, D | 1 |
| Meikle, PJ | 1 |
| Brooks, DA | 1 |
1 other study available for butyric acid and Glycogen Storage Disease Type II
| Article | Year |
|---|---|
Stabilising normal and mis-sense variant alpha-glucosidase.
Topics: alpha-Glucosidases; Animals; Butyric Acid; CHO Cells; Cricetinae; Cricetulus; Enzyme Stability; Fibr | 2006 |