gentamicin has been researched along with Mucopolysaccharidosis I in 6 studies
Gentamicins: A complex of closely related aminoglycosides obtained from MICROMONOSPORA purpurea and related species. They are broad-spectrum antibiotics, but may cause ear and kidney damage. They act to inhibit PROTEIN BIOSYNTHESIS.
Mucopolysaccharidosis I: Systemic lysosomal storage disease caused by a deficiency of alpha-L-iduronidase (IDURONIDASE) and characterized by progressive physical deterioration with urinary excretion of DERMATAN SULFATE and HEPARAN SULFATE. There are three recognized phenotypes representing a spectrum of clinical severity from severe to mild: Hurler syndrome, Hurler-Scheie syndrome and Scheie syndrome (formerly mucopolysaccharidosis V). Symptoms may include DWARFISM; hepatosplenomegaly; thick, coarse facial features with low nasal bridge; corneal clouding; cardiac complications; and noisy breathing.
Excerpt | Relevance | Reference |
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"Mucopolysaccharidosis type I Hurler syndrome (MPS IH) is a severe lysosomal storage disorder caused by alpha-l-iduronidase (IDUA) deficiency." | 1.72 | Effects of gentamicin inducing readthrough premature stop Codons: A study of alpha-L-iduronidase nonsense variants in COS-7 Cells. ( Champattanachai, V; Dejkhamron, P; Ketudat-Cairns, JR; Ngiwsara, L; Sawangareetrakul, P; Svasti, J; Tim-Aroon, T; Wattanasirichaigoon, D, 2022) |
"We found that a Hurler syndrome fibroblast cell line heterozygous for the IDUA stop mutations Q70X and W402X showed a significant increase in alpha-L-iduronidase activity when cultured in the presence of gentamicin, resulting in the restoration of 2." | 1.31 | Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation. ( Bedwell, DM; Brooks, DA; Hopwood, JJ; Keeling, KM; Li, P; Thompson, JN, 2001) |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 0 (0.00) | 18.7374 |
1990's | 0 (0.00) | 18.2507 |
2000's | 4 (66.67) | 29.6817 |
2010's | 1 (16.67) | 24.3611 |
2020's | 1 (16.67) | 2.80 |
Authors | Studies |
---|---|
Ngiwsara, L | 1 |
Sawangareetrakul, P | 1 |
Wattanasirichaigoon, D | 1 |
Tim-Aroon, T | 1 |
Dejkhamron, P | 1 |
Champattanachai, V | 1 |
Ketudat-Cairns, JR | 1 |
Svasti, J | 1 |
Nudelman, I | 1 |
Glikin, D | 1 |
Smolkin, B | 1 |
Hainrichson, M | 1 |
Belakhov, V | 1 |
Baasov, T | 1 |
Hein, LK | 1 |
Bawden, M | 1 |
Muller, VJ | 1 |
Sillence, D | 1 |
Hopwood, JJ | 2 |
Brooks, DA | 2 |
Keeling, KM | 2 |
Li, P | 1 |
Thompson, JN | 1 |
Bedwell, DM | 2 |
Stephenson, J | 1 |
6 other studies available for gentamicin and Mucopolysaccharidosis I
Article | Year |
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Effects of gentamicin inducing readthrough premature stop Codons: A study of alpha-L-iduronidase nonsense variants in COS-7 Cells.
Topics: Animals; Chlorocebus aethiops; Codon, Nonsense; Codon, Terminator; COS Cells; Gentamicins; Iduronida | 2022 |
Repairing faulty genes by aminoglycosides: development of new derivatives of geneticin (G418) with enhanced suppression of diseases-causing nonsense mutations.
Topics: Aminoglycosides; Animals; Codon, Nonsense; Cystic Fibrosis; Drug Design; Genetic Diseases, Inborn; G | 2010 |
alpha-L-iduronidase premature stop codons and potential read-through in mucopolysaccharidosis type I patients.
Topics: Animals; Anti-Bacterial Agents; CHO Cells; Codon, Nonsense; Cricetinae; Gentamicins; Humans; Iduroni | 2004 |
Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation.
Topics: Anti-Bacterial Agents; Cell Line; Codon, Terminator; Dose-Response Relationship, Drug; Fibroblasts; | 2001 |
Antibiotics show promise as therapy for genetic disorders.
Topics: Animals; Anti-Bacterial Agents; Cystic Fibrosis; Gentamicins; Humans; Mice; Mice, Mutant Strains; Mu | 2001 |
Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation system.
Topics: Amikacin; Aminoglycosides; Animals; Anti-Bacterial Agents; Cells, Cultured; Codon, Nonsense; DNA, Co | 2002 |