Huntingtin Modulates Transcription, Occupies Gene Promoters<i>In Vivo</i>, and Binds Directly to DNA in a Polyglutamine-Dependent Manner

Benn, Caroline; Sun, Tingting; Sadri‐Vakili, Ghazaleh; McFarland, Karen N.; DiRocco, Derek P.; Yohrling, George J.; Clark, Tim W.; Bouzou, Bérengère; Jang-Ho, J.

doi:10.1523/jneurosci.2126-08.2008

Cited by 184 publications

(148 citation statements)

References 59 publications

(93 reference statements)

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“…20 This cell model of HD has been extensively used for identifying molecular alterations in HD. [41][42][43][44][45][46][47][48] Cell culture and transfection. (Cat No.…”

Section: O N O T D I S T R I B U T Ementioning

confidence: 99%

Micro RNA -214,-150,-146a and-125b targetHuntingtingene

Sinha¹,

Ghose²,

Bhattarcharyya³

2011

RNA Biology

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“…20 This cell model of HD has been extensively used for identifying molecular alterations in HD. [41][42][43][44][45][46][47][48] Cell culture and transfection. (Cat No.…”

Section: O N O T D I S T R I B U T Ementioning

confidence: 99%

Micro RNA -214,-150,-146a and-125b targetHuntingtingene

Sinha¹,

Ghose²,

Bhattarcharyya³

2011

RNA Biology

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“…Many of the REST target genes have been shown to be down-regulated in HD (15). Moreover, there are reports indicating an overall increase in REST expression in various models of HD (25,36,39). The importance of REST in regulating HD pathogenesis inspired us to investigate whether HIPPI-mediated up-regulation of REST plays any role in HD.…”

Section: Discussionmentioning

confidence: 99%

Regulation of RE1 Protein Silencing Transcription Factor (REST) Expression by HIP1 Protein Interactor (HIPPI)

Datta

Bhattacharyya

2011

Journal of Biological Chemistry

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Background: HIPPI, along with its molecular partner HIP1, can regulate transcription of the caspase-1 gene. Results: HIPPI binds to the promoter of REST and increases its expression in neuronal and non-neuronal cells and in a Huntington disease cell model. Conclusion: HIPPI is a novel transcription regulator of REST. Significance: This study provides a novel mechanistic interpretation of HD pathogenesis through HIPPI-mediated transcriptional regulation of REST.

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“…For example, mutant and wild-type huntingtin have been shown to have direct DNAbinding sites, and these sites are different. 99 Therefore, mutant huntingtin could perturb normal transcription by preventing or modulating the binding of normal transcription factors to DNA. Alternatively, there is evidence that transcriptional deregulation could be due to the presence of mRNAs carrying the expansions of CAG codons coding for the poly-Q stretches.…”

Section: ¡12mentioning

confidence: 99%

Prions, amyloids, and RNA: Pieces of a puzzle

Nizhnikov

Antonets

Bondarev

et al. 2016

Prion

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ABSTRACT. Amyloids are protein aggregates consisting of fibrils rich in b-sheets. Growth of amyloid fibrils occurs by the addition of protein molecules to the tip of an aggregate with a concurrent change of a conformation. Thus, amyloids are self-propagating protein conformations. In certain cases these conformations are transmissible / infectious; they are known as prions. Initially, amyloids were discovered as pathological extracellular deposits occurring in different tissues and organs. To date, amyloids and prions have been associated with over 30 incurable diseases in humans and animals. However, a number of recent studies demonstrate that amyloids are also functionally involved in a variety of biological processes, from biofilm formation by bacteria, to long-term memory in animals. Interestingly, amyloid-forming proteins are highly overrepresented among cellular factors engaged in all stages of mRNA life cycle: from transcription and translation, to storage and degradation. Here we review rapidly accumulating data on functional and pathogenic amyloids associated with mRNA processing, and discuss possible significance of prion and amyloid networks in the modulation of key cellular functions.

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Huntingtin Modulates Transcription, Occupies Gene PromotersIn Vivo, and Binds Directly to DNA in a Polyglutamine-Dependent Manner

Abstract: Transcriptional dysregulation is a central pathogenic mechanism in

Cited by 184 publications

References 59 publications

Micro RNA -214,-150,-146a and-125b targetHuntingtingene

Micro RNA -214,-150,-146a and-125b targetHuntingtingene

Regulation of RE1 Protein Silencing Transcription Factor (REST) Expression by HIP1 Protein Interactor (HIPPI)

Prions, amyloids, and RNA: Pieces of a puzzle

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