Page last updated: 2024-10-27

gentamicin and Mucopolysaccharidosis I

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.

Research Excerpts

ExcerptRelevanceReference
"Mucopolysaccharidosis type I Hurler syndrome (MPS IH) is a severe lysosomal storage disorder caused by alpha-l-iduronidase (IDUA) deficiency."1.72Effects 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."1.31Gentamicin-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)

Research

Studies (6)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's4 (66.67)29.6817
2010's1 (16.67)24.3611
2020's1 (16.67)2.80

Authors

AuthorsStudies
Ngiwsara, L1
Sawangareetrakul, P1
Wattanasirichaigoon, D1
Tim-Aroon, T1
Dejkhamron, P1
Champattanachai, V1
Ketudat-Cairns, JR1
Svasti, J1
Nudelman, I1
Glikin, D1
Smolkin, B1
Hainrichson, M1
Belakhov, V1
Baasov, T1
Hein, LK1
Bawden, M1
Muller, VJ1
Sillence, D1
Hopwood, JJ2
Brooks, DA2
Keeling, KM2
Li, P1
Thompson, JN1
Bedwell, DM2
Stephenson, J1

Other Studies

6 other studies available for gentamicin and Mucopolysaccharidosis I

ArticleYear
Effects of gentamicin inducing readthrough premature stop Codons: A study of alpha-L-iduronidase nonsense variants in COS-7 Cells.
    Biochemical and biophysical research communications, 2022, 12-25, Volume: 636, Issue:Pt 1

    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.
    Bioorganic & medicinal chemistry, 2010, Jun-01, Volume: 18, Issue:11

    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.
    Journal of molecular biology, 2004, Apr-30, Volume: 338, Issue:3

    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.
    Human molecular genetics, 2001, Feb-01, Volume: 10, Issue:3

    Topics: Anti-Bacterial Agents; Cell Line; Codon, Terminator; Dose-Response Relationship, Drug; Fibroblasts;

2001
Antibiotics show promise as therapy for genetic disorders.
    JAMA, 2001, Apr-25, Volume: 285, Issue:16

    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.
    Journal of molecular medicine (Berlin, Germany), 2002, Volume: 80, Issue:6

    Topics: Amikacin; Aminoglycosides; Animals; Anti-Bacterial Agents; Cells, Cultured; Codon, Nonsense; DNA, Co

2002