We are analyzing https://link.springer.com/article/10.1007/s00401-013-1200-z.

Title:
C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci | Acta Neuropathologica
Description:
An expanded GGGGCC repeat in a non-coding region of the C9orf72 gene is a common cause of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis. Non-coding repeat expansions may cause disease by reducing the expression level of the gene they reside in, by producing toxic aggregates of repeat RNA termed RNA foci, or by producing toxic proteins generated by repeat-associated non-ATG translation. We present the first definitive report of C9orf72 repeat sense and antisense RNA foci using a series of C9FTLD cases, and neurodegenerative disease and normal controls. A sensitive and specific fluorescence in situ hybridisation protocol was combined with protein immunostaining to show that both sense and antisense foci were frequent, specific to C9FTLD, and present in neurons of the frontal cortex, hippocampus and cerebellum. High-resolution imaging also allowed accurate analyses of foci number and subcellular localisation. RNA foci were most abundant in the frontal cortex, where 51 % of neurons contained foci. RNA foci also occurred in astrocytes, microglia and oligodendrocytes but to a lesser degree than in neurons. RNA foci were observed in both TDP-43- and p62-inclusion bearing neurons, but not at a greater frequency than expected by chance. RNA foci abundance in the frontal cortex showed a significant inverse correlation with age at onset of disease. These data establish that sense and antisense C9orf72 repeat RNA foci are a consistent and specific feature of C9FTLD, providing new insight into the pathogenesis of C9FTLD.
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Keywords {🔍}

foci, rna, sense, antisense, cftld, fig, cases, neurons, frontal, cortex, article, corf, case, neuron, hippocampus, google, scholar, brain, pubmed, repeat, cerebellum, homozygous, cell, inclusions, fish, heterozygous, cas, tdp, shown, frontotemporal, neuronal, disease, quantification, specific, observed, cytoplasmic, regions, number, nucleus, present, dementia, type, probe, image, granule, red, ggggcc, age, expansion, performed,

Topics {✒️}

chromosome 9p21-linked als-ftd chromosome 9p-linked ftd article download pdf cac-tip-ptd-p01 frontotemporal lobar degeneration depth clinico-pathological examination aggregating dipeptide-repeat proteins amyotrophic lateral sclerosis 7 μm-thick paraffin sections ipsc-derived human neurons high-resolution confocal imaging 2′-o-methyl rna probes 2′-o-methyl rna probe atg-initiated translation directed high-stringency wash solution c9orf72 expansion carriers multiple neurodegenerative syndromes c9orf72 repeat expansion hexanucleotide repeat expansion tdp-43-positive cytoplasmic inclusions c9orf72 hexanucleotide repeat full access privacy choices/manage cookies related subjects c9orf72-linked ftld reveals total foci oligodendrocyte marker caii c9orf72 ggggcc repeat article mizielinska cohn-hokke pe high-resolution imaging g-quadruplex structure [11] p62-inclusion bearing neurons expanded ggggcc repeat phenomenon termed repeat expanded repeat rna original author c9orf72 repeat sense high melting temperature producing toxic aggregates 8 mg/ml trna neuronal rna foci coding repeat expansions author information authors graphpad prism software stranded structure termed small size pre-hybridisation solution toxic rna hypothesis research uk

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         headline:C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci
         description:An expanded GGGGCC repeat in a non-coding region of the C9orf72 gene is a common cause of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis. Non-coding repeat expansions may cause disease by reducing the expression level of the gene they reside in, by producing toxic aggregates of repeat RNA termed RNA foci, or by producing toxic proteins generated by repeat-associated non-ATG translation. We present the first definitive report of C9orf72 repeat sense and antisense RNA foci using a series of C9FTLD cases, and neurodegenerative disease and normal controls. A sensitive and specific fluorescence in situ hybridisation protocol was combined with protein immunostaining to show that both sense and antisense foci were frequent, specific to C9FTLD, and present in neurons of the frontal cortex, hippocampus and cerebellum. High-resolution imaging also allowed accurate analyses of foci number and subcellular localisation. RNA foci were most abundant in the frontal cortex, where 51 % of neurons contained foci. RNA foci also occurred in astrocytes, microglia and oligodendrocytes but to a lesser degree than in neurons. RNA foci were observed in both TDP-43- and p62-inclusion bearing neurons, but not at a greater frequency than expected by chance. RNA foci abundance in the frontal cortex showed a significant inverse correlation with age at onset of disease. These data establish that sense and antisense C9orf72 repeat RNA foci are a consistent and specific feature of C9FTLD, providing new insight into the pathogenesis of C9FTLD.
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      headline:C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci
      description:An expanded GGGGCC repeat in a non-coding region of the C9orf72 gene is a common cause of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis. Non-coding repeat expansions may cause disease by reducing the expression level of the gene they reside in, by producing toxic aggregates of repeat RNA termed RNA foci, or by producing toxic proteins generated by repeat-associated non-ATG translation. We present the first definitive report of C9orf72 repeat sense and antisense RNA foci using a series of C9FTLD cases, and neurodegenerative disease and normal controls. A sensitive and specific fluorescence in situ hybridisation protocol was combined with protein immunostaining to show that both sense and antisense foci were frequent, specific to C9FTLD, and present in neurons of the frontal cortex, hippocampus and cerebellum. High-resolution imaging also allowed accurate analyses of foci number and subcellular localisation. RNA foci were most abundant in the frontal cortex, where 51 % of neurons contained foci. RNA foci also occurred in astrocytes, microglia and oligodendrocytes but to a lesser degree than in neurons. RNA foci were observed in both TDP-43- and p62-inclusion bearing neurons, but not at a greater frequency than expected by chance. RNA foci abundance in the frontal cortex showed a significant inverse correlation with age at onset of disease. These data establish that sense and antisense C9orf72 repeat RNA foci are a consistent and specific feature of C9FTLD, providing new insight into the pathogenesis of C9FTLD.
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         FTD
         ALS
         RNA foci
         Antisense
          C9orf72
         Pathology
         Neurosciences
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      name:Department of Neurodegenerative Disease, UCL Institute of Neurology, London, UK
      name:MRC Centre for Neuromuscular Disease, UCL Institute of Neurology, London, UK
      name:Department of Neurodegenerative Disease, UCL Institute of Neurology, London, UK

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