Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblasts (Q28261807)

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Jinhua Wang

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Cheng-Wei T Chang

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Tristan Coady

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Christian L Lorson

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

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language of work or name

English

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publication date

November 2006

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published in

Human Genetics

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volume

120

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issue

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page(s)

589-601

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cites work

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12 December 2020

Riluzole attenuates spinal muscular atrophy disease progression in a mouse model.

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12 December 2020

An update of the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA).

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12 December 2020

Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein

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12 December 2020

A novel nuclear structure containing the survival of motor neurons protein

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12 December 2020

The neurobiology of childhood spinal muscular atrophy.

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12 December 2020

A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy.

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12 December 2020

Homozygous exon 7 deletion of the SMN centromeric gene (SMN2): a potential susceptibility factor for adult-onset lower motor neuron disease

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12 December 2020

A mouse model for spinal muscular atrophy

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12 December 2020

SMN oligomerization defect correlates with spinal muscular atrophy severity

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12 December 2020

SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN

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12 December 2020

Aminoglycoside-mediated suppression of nonsense mutations in late infantile neuronal ceroid lipofuscinosis

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12 December 2020

Expression study of survival motor neuron gene in human fetal tissues.

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12 December 2020

An in vivo reporter system for measuring increased inclusion of exon 7 in SMN2 mRNA: potential therapy of SMA

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12 December 2020

Readthrough of dystrophin stop codon mutations induced by aminoglycosides

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12 December 2020

The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophy

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12 December 2020

The role of the SMN gene in proximal spinal muscular atrophy

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12 December 2020

Phenylbutyrate increases SMN expression in vitro: relevance for treatment of spinal muscular atrophy

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12 December 2020

Valproic acid increases the SMN2 protein level: a well-known drug as a potential therapy for spinal muscular atrophy

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12 December 2020

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

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12 December 2020

The role of histone acetylation in SMN gene expression.

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12 December 2020

Indoprofen upregulates the survival motor neuron protein through a cyclooxygenase-independent mechanism

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12 December 2020

Lentivector-mediated SMN replacement in a mouse model of spinal muscular atrophy.

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12 December 2020

Correlation between severity and SMN protein level in spinal muscular atrophy

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12 December 2020

Coiled bodies and gems: Janus or gemini?

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12 December 2020

Gentamicin treatment of Duchenne and Becker muscular dystrophy due to nonsense mutations

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12 December 2020

Aclarubicin treatment restores SMN levels to cells derived from type I spinal muscular atrophy patients

1 reference

12 December 2020

Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice

1 reference

12 December 2020

Spinal muscular atrophy

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12 December 2020

Application of glycodiversification: expedient synthesis and antibacterial evaluation of a library of kanamycin B analogues

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12 December 2020

Animal models of spinal muscular atrophy

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12 December 2020

Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy

1 reference

12 December 2020

Disruption of an SF2/ASF-dependent exonic splicing enhancer in SMN2 causes spinal muscular atrophy in the absence of SMN1.

1 reference

12 December 2020

Modulation of SMN nuclear foci and cytoplasmic localization by its C-terminus

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12 December 2020

Treatment of spinal muscular atrophy by sodium butyrate

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12 December 2020

Pyranmycins, a novel class of aminoglycosides with improved acid stability: the SAR of D-pyranoses on ring III of pyranmycin.

1 reference

12 December 2020

The survival motor neuron protein in spinal muscular atrophy

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12 December 2020

The SMN complex, an assemblyosome of ribonucleoproteins

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12 December 2020

A non-sequence-specific requirement for SMN protein activity: the role of aminoglycosides in inducing elevated SMN protein levels

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12 December 2020

Application of the synthetic aminosugars for glycodiversification: synthesis and antimicrobial studies of pyranmycin

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12 December 2020

Phenylbutyrate increases SMN gene expression in spinal muscular atrophy patients

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

Valproic acid increases SMN levels in spinal muscular atrophy patient cells.

1 reference

12 December 2020

Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization.

1 reference

12 December 2020

Diverse small-molecule modulators of SMN expression found by high-throughput compound screening: early leads towards a therapeutic for spinal muscular atrophy.

1 reference

12 December 2020

Glycodiversification for the optimization of the kanamycin class aminoglycosides

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12 December 2020

Identification and characterization of a spinal muscular atrophy-determining gene

1 reference

12 December 2020

Nuclear gems and Cajal (coiled) bodies in fetal tissues: nucleolar distribution of the spinal muscular atrophy protein, SMN

1 reference

12 December 2020

The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation.

1 reference

12 December 2020

Hydroxyurea enhances SMN2 gene expression in spinal muscular atrophy cells

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12 December 2020

A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy

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12 December 2020

Correction of ATM gene function by aminoglycoside-induced read-through of premature termination codons

1 reference

12 December 2020

Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.

1 reference

12 December 2020

A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2.

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12 December 2020