(Q35661233)

9 August 2017

title

The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse (English)

1 reference

9 August 2017

Dan Wang

1

1 reference

9 August 2017

Valery Belakhov

2

1 reference

9 August 2017

Jeyakumar Kandasamy

3

1 reference

9 August 2017

Timor Baasov

4

1 reference

9 August 2017

Su-Chen Li

5

1 reference

9 August 2017

Yu-Teh Li

6

1 reference

9 August 2017

David M Bedwell

7

1 reference

9 August 2017

Kim M Keeling

8

1 reference

9 August 2017

publication date

19 October 2011

1 reference

Molecular Genetics and Metabolism

9 August 2017

published in

1 reference

9 August 2017

volume

105

1 reference

9 August 2017

issue

1

1 reference

9 August 2017

page(s)

116-125

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Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases

9 August 2017

cites work

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Genistein reduces glycosaminoglycan levels in a mouse model of mucopolysaccharidosis type II

12 July 2018

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Nonaminoglycoside compounds induce readthrough of nonsense mutations

12 July 2018

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Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

12 July 2018

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Development of novel aminoglycoside (NB54) with reduced toxicity and enhanced suppression of disease-causing premature stop mutations

12 July 2018

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12 July 2018

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Lysosomal disorders: from storage to cellular damage

12 July 2018

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Genetic analysis of interactions with eukaryotic rRNA identify the mitoribosome as target in aminoglycoside ototoxicity

12 July 2018

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12 July 2018

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The clinical outcome of Hurler syndrome after stem cell transplantation.

12 July 2018

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Eukaryotic ribosomal RNA determinants of aminoglycoside resistance and their role in translational fidelity.

12 July 2018

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PTC124 targets genetic disorders caused by nonsense mutations

12 July 2018

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12 July 2018

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Molecular aspects of renal handling of aminoglycosides and strategies for preventing the nephrotoxicity

12 July 2018

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The cell biology of lysosomal storage disorders

12 July 2018

1 reference

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Secondary accumulation of gangliosides in lysosomal storage disorders

12 July 2018

1 reference

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Readthrough of dystrophin stop codon mutations induced by aminoglycosides

12 July 2018

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Megalin and cubilin: multifunctional endocytic receptors

12 July 2018

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UAG readthrough in mammalian cells: effect of upstream and downstream stop codon contexts reveal different signals

12 July 2018

1 reference

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Structural origins of aminoglycoside specificity for prokaryotic ribosomes

12 July 2018

1 reference

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Structure of a eukaryotic decoding region A-site RNA

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Bone marrow transplantation for globoid cell leukodystrophy, adrenoleukodystrophy, metachromatic leukodystrophy, and Hurler syndrome

12 July 2018

1 reference

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Aminoglycosides: nephrotoxicity

12 July 2018

1 reference

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Genotype-phenotype correlations in mucopolysaccharidosis type I using enzyme kinetics, immunoquantification and in vitro turnover studies.

12 July 2018

1 reference

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Murine MPS I: insights into the pathogenesis of Hurler syndrome.

12 July 2018

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A dual-luciferase reporter system for studying recoding signals

12 July 2018

1 reference

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rRNA chemical groups required for aminoglycoside binding.

12 July 2018

1 reference

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Murine mucopolysaccharidosis type I: targeted disruption of the murine alpha-L-iduronidase gene

12 July 2018

1 reference

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RNA sequence determinants for aminoglycoside binding to an A-site rRNA model oligonucleotide

12 July 2018

1 reference

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Context effects on misreading and suppression at UAG codons in human cells.

12 July 2018

1 reference

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On the relationship between preferred termination codon contexts and nonsense suppression in human cells

12 July 2018

1 reference

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Effect of osmotic blood-brain barrier disruption on gentamicin penetration into the cerebrospinal fluid and brains of normal rabbits

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Pharmacologic evaluation of gentamicin in newborn infants

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Gentamicin penetration into cerebrospinal fluid in experimental Haemophilus influenzae meningitis

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Long-term protection of polyaspartic acid in experimental gentamicin nephrotoxicity

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Immunoquantification and enzyme kinetics of alpha-L-iduronidase in cultured fibroblasts from normal controls and mucopolysaccharidosis type I patients

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Aminoglycosides redesign strategies for improved antibiotics and compounds for treatment of human genetic diseases

12 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Repairing faulty genes by aminoglycosides: development of new derivatives of geneticin (G418) with enhanced suppression of diseases-causing nonsense mutations

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Antioxidant gene therapy can protect hearing and hair cells from ototoxicity

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation system

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Antioxidants attenuate gentamicin-induced free radical formation in vitro and ototoxicity in vivo: D-methionine is a potential protectant.

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Mucopolysaccharidosis type I: identification of 8 novel mutations and determination of the frequency of the two common alpha-L-iduronidase mutations (W402X and Q70X) among European patients.

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3253910

Diphenylamine-aniline-phosphoric acid reagent, a versatile spray reagent for revealing glycoconjugates on thin-layer chromatography plates

25 September 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.10.005

Sequence specificity of aminoglycoside-induced stop condon readthrough: potential implications for treatment of Duchenne muscular dystrophy

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.10.005

The occurrence of GM4 and GM2 in erythrocytes from inbred strains of mice

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.10.005

Redesign of aminoglycosides for treatment of human genetic diseases caused by premature stop mutations

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.10.005

Intrathecal gentamicin in treatment of ventriculitis in children

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2011.10.005

10.1016/J.YMGME.2011.10.005

7 January 2021

Identifiers

DOI

1 reference

9 August 2017

1 reference

9 August 2017

1 reference