A structural approach to trinucleotide expansion diseases. (Q30329219)

18 June 2017

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title

A structural approach to trinucleotide expansion diseases. (English)

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18 June 2017

Masino L

1

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18 June 2017

Pastore A

series ordinal

2

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18 June 2017

publication date

1 October 2001

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18 June 2017

published in

Brain Research Bulletin

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18 June 2017

volume

56

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18 June 2017

issue

3-4

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18 June 2017

page(s)

183-189

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Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia

18 June 2017

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Dissecting FMR1, the protein responsible for fragile X syndrome, in its structural and functional domains

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Random coil conformation for extended polyglutamine stretches in aqueous soluble monomeric peptides.

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Structure of polyglutamine

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FMR1 protein: conserved RNP family domains and selective RNA binding

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Accessory factor-bZIP-DNA interactions

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A novel RNA-binding nuclear protein that interacts with the fragile X mental retardation (FMR1) protein

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7 January 2021

Purified recombinant Fmrp exhibits selective RNA binding as an intrinsic property of the fragile X mental retardation protein

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7 January 2021

Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

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Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

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Loss of normal huntingtin function: new developments in Huntington's disease research

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Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae.

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Isolation of an FMRP-associated messenger ribonucleoprotein particle and identification of nucleolin and the fragile X-related proteins as components of the complex

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7 January 2021

Crystal structure of a dimeric chymotrypsin inhibitor 2 mutant containing an inserted glutamine repeat

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Crystal structure of Escherichia coli CyaY protein reveals a previously unidentified fold for the evolutionarily conserved frataxin family

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Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I.

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The fragile X mental retardation protein is associated with poly(A)+ mRNA in actively translating polyribosomes

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7 January 2021

Fourteen and counting: unraveling trinucleotide repeat diseases

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7 January 2021

The FMR-1 protein is cytoplasmic, most abundant in neurons and appears normal in carriers of a fragile X premutation

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7 January 2021

Crystal structure of human frataxin

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FMRP associates with polyribosomes as an mRNP, and the I304N mutation of severe fragile X syndrome abolishes this association

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Fragile X mental retardation protein: nucleocytoplasmic shuttling and association with somatodendritic ribosomes

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7 January 2021

Friedreich's ataxia protein: phylogenetic evidence for mitochondrial dysfunction

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7 January 2021

The KH domain occurs in a diverse set of RNA-binding proteins that include the antiterminator NusA and is probably involved in binding to nucleic acid

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7 January 2021

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7 January 2021

Understanding the molecular basis of fragile X syndrome

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7 January 2021

Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: relevance to diseases of the nervous system

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7 January 2021

Aberrant protein deposition and neurological disease

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7 January 2021

Evidence that fragile X mental retardation protein is a negative regulator of translation

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7 January 2021

Sequence-specific RNA binding by a Nova KH domain: implications for paraneoplastic disease and the fragile X syndrome

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7 January 2021

Amyloid-like inclusions in Huntington's disease.

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7 January 2021

Poly-L-glutamine forms cation channels: relevance to the pathogenesis of the polyglutamine diseases

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7 January 2021

New tubular single-stranded helix of poly-L-amino acids suggested by molecular mechanics calculations: I. Homopolypeptides in isolated environments

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7 January 2021

Three-dimensional structure and stability of the KH domain: molecular insights into the fragile X syndrome

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7 January 2021

The solution structure of the first KH domain of FMR1, the protein responsible for the fragile X syndrome

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7 January 2021

Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin

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7 January 2021

Reversing neurodegeneration: a promise unfolds

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7 January 2021

Molecular pathogenesis of Friedreich ataxia

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7 January 2021

Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold

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7 January 2021

Trinucleotide repeat DNA structures: dynamic mutations from dynamic DNA

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7 January 2021

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Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

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7 January 2021

Glutamine repeats and inherited neurodegenerative diseases: molecular aspects

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7 January 2021

Absence of expression of the FMR-1 gene in fragile X syndrome

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7 January 2021

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

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7 January 2021

A highly conserved protein family interacting with the fragile X mental retardation protein (FMRP) and displaying selective interactions with FMRP-related proteins FXR1P and FXR2P

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7 January 2021

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

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7 January 2021

Peptide models for inherited neurodegenerative disorders: conformation and aggregation properties of long polyglutamine peptides with and without interruptions

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7 January 2021

The protein product of the fragile X gene, FMR1, has characteristics of an RNA-binding protein

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7 January 2021

Essential role for KH domains in RNA binding: impaired RNA binding by a mutation in the KH domain of FMR1 that causes fragile X syndrome

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7 January 2021

Specific sequences in the fragile X syndrome protein FMR1 and the FXR proteins mediate their binding to 60S ribosomal subunits and the interactions among them

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7 January 2021

Folding of oligoglutamines: a theoretical approach based upon thermodynamics and molecular mechanics.

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7 January 2021

Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases

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7 January 2021

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

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7 January 2021

Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndrome

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7 January 2021

Polyglutamine diseases: protein cleavage and aggregation.

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7 January 2021

Glutamine repeats and neurodegeneration

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7 January 2021

10.1016/S0361-9230(01)00582-2

Identifiers

DOI

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18 June 2017

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