Interconverting conformations of slipped-DNA junctions formed by trinucleotide repeats affect repair outcome.

Abstract:

:Expansions of (CTG)·(CAG) repeated DNAs are the mutagenic cause of 14 neurological diseases, likely arising through the formation and processing of slipped-strand DNAs. These transient intermediates of repeat length mutations are formed by out-of-register mispairing of repeat units on complementary strands. The three-way slipped-DNA junction, at which the excess repeats slip out from the duplex, is a poorly understood feature common to these mutagenic intermediates. Here, we reveal that slipped junctions can assume a surprising number of interconverting conformations where the strand opposite the slip-out either is fully base paired or has one or two unpaired nucleotides. These unpaired nucleotides can also arise opposite either of the nonslipped junction arms. Junction conformation can affect binding by various structure-specific DNA repair proteins and can also alter correct nick-directed repair levels. Junctions that have the potential to contain unpaired nucleotides are repaired with a significantly higher efficiency than constrained fully paired junctions. Surprisingly, certain junction conformations are aberrantly repaired to expansion mutations: misdirection of repair to the non-nicked strand opposite the slip-out leads to integration of the excess slipped-out repeats rather than their excision. Thus, slipped-junction structure can determine whether repair attempts lead to correction or expansion mutations.

journal_name

Biochemistry

journal_title

Biochemistry

authors

Slean MM,Reddy K,Wu B,Nichol Edamura K,Kekis M,Nelissen FH,Aspers RL,Tessari M,Schärer OD,Wijmenga SS,Pearson CE

doi

10.1021/bi301369b

subject

Has Abstract

pub_date

2013-02-05 00:00:00

pages

773-85

issue

5

eissn

0006-2960

issn

1520-4995

journal_volume

52

pub_type

杂志文章
  • Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C.

    abstract::The abnormal isoform of the scrapie prion protein PrPSc is both a host-derived protein and a component of the infectious agent causing scrapie. PrPSc and the normal cellular isoform PrPC have different physical properties that apparently arise from a posttranslational event. Both PrP isoforms are covalently modified a...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00474a028

    authors: Stahl N,Borchelt DR,Prusiner SB

    更新日期:1990-06-05 00:00:00

  • WIN 17317-3, a new high-affinity probe for voltage-gated sodium channels.

    abstract::The iminodihydroquinoline WIN 17317-3 was previously shown to inhibit selectively the voltage-gated potassium channels, K(v)1.3 and K(v)1.4 [Hill, R. J., et al. (1995) Mol. Pharmacol. 48, 98-104; Nguyen, A., et al. (1996) Mol. Pharmacol. 50, 1672-1679]. Since these channels are found in brain, radiolabeled WIN 17317-3...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi990336p

    authors: Wanner SG,Glossmann H,Knaus HG,Baker R,Parsons W,Rupprecht KM,Brochu R,Cohen CJ,Schmalhofer W,Smith M,Warren V,Garcia ML,Kaczorowski GJ

    更新日期:1999-08-24 00:00:00

  • A phosphotyrosyl mimetic peptide reverses impairment of insulin-stimulated translocation of GLUT4 caused by overexpression of PTP1B in rat adipose cells.

    abstract::Biological actions of insulin are initiated by activation of the insulin receptor tyrosine kinase. Protein tyrosine phosphatases (PTPases) PTP1B and PTPalpha are known to dephosphorylate the insulin receptor and may contribute to insulin resistance in diseases such as diabetes. We previously reported that overexpressi...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi9816103

    authors: Chen H,Cong LN,Li Y,Yao ZJ,Wu L,Zhang ZY,Burke TR Jr,Quon MJ

    更新日期:1999-01-05 00:00:00

  • Genome-Wide Mapping of Oxidative DNA Damage via Engineering of 8-Oxoguanine DNA Glycosylase.

    abstract::The occurrence of 8-oxo-7,8-dihydroguanine (OG) in the genome, as one of the major DNA oxidative damages, has been implicated in an array of biological processes, ranging from mutagenesis to transcriptional regulation. Genome-wide mapping of oxidative damages could shed light on the underlying cellular mechanism. In t...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/acs.biochem.9b00782

    authors: Fang Y,Zou P

    更新日期:2020-01-14 00:00:00

  • Association of the polyadenylate segment of messenger RNA with other polynucleotide sequences in mouse sarcoma 180 polyribosomes.

    abstract::Limited digestion of polysomal RNA with pancreatic ribonuclease releases a structure consisting of poly(A) associated with other polyribonucleotide sequences. This complex can be purified by oligo(dT)-cellulose chromatography. Heating for formamide treatment causes the dissociation of fragments free of poly(A) from th...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00686a024

    authors: Jeffery WR,Brawerman G

    更新日期:1975-07-29 00:00:00

  • Escherichia coli phosphoenolpyruvate dependent phosphotransferase system. Copurification of HPr and alpha 1-6 glucan.

    abstract::A rapid, high-yield procedure has been developed for the purification of HPr from the Escherichia coli phosphoenolpyruvate dependent phosphotransferase system. During this procedure, the protein copurifies with a 2500-dalton homopolysaccharide which we have identified as alpha 1-6 glucan. The results of steady-state k...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00581a013

    authors: Dooijewaard G,Roossien FF,Robillard GT

    更新日期:1979-07-10 00:00:00

  • Inhibitors bound to Ca(2+)-free sarcoplasmic reticulum Ca(2+)-ATPase lock its transmembrane region but not necessarily its cytosolic region, revealing the flexibility of the loops connecting transmembrane and cytosolic domains.

    abstract::Ca2+-free crystals of sarcoplasmic reticulum Ca2+-ATPase have, up until now, been obtained in the presence of inhibitors such as thapsigargin (TG), bound to the transmembrane region of this protein. Here, we examined the consequences of such binding for the protein. We found that, after TG binding, an active site liga...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi701855r

    authors: Montigny C,Picard M,Lenoir G,Gauron C,Toyoshima C,Champeil P

    更新日期:2007-12-25 00:00:00

  • Solution structure of oxidized cytochrome c6 from the green alga Monoraphidium braunii.

    abstract::Cytochrome c6 from Monoraphidium braunii, an 89-amino acid electron transfer protein, has been investigated by NMR in solution, in its oxidized form, at pH 7 and 300 K. By using a combination of COSY, TOCSY, and NOESY experiments, 84% of the proton resonances have been assigned. A total of 1668 experimental NOE constr...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi972765y

    authors: Banci L,Bertini I,De la Rosa MA,Koulougliotis D,Navarro JA,Walter O

    更新日期:1998-04-07 00:00:00

  • Decay of activated Bacillus subtilis pho response regulator, PhoP approximately P, involves the PhoR approximately P intermediate.

    abstract::PhoR of Bacillus subtilis is a histidine sensor-kinase belonging to the family of two-component signal transduction systems. PhoR is responsible for processing the phosphate-starvation signal and providing phosphate input to regulate the level of phosphorylated response regulator, PhoP, which activates/represses Pho r...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi990658t

    authors: Shi L,Liu W,Hulett FM

    更新日期:1999-08-03 00:00:00

  • Inactivation of factor VIII by factor IXa.

    abstract::Factor VIII (FVIII) is the nonproteolytic cofactor for FIXa in the tenase complex of blood coagulation. FVIII is proteolytically activated by thrombin and FXa in vitro to form a heterotrimer with full procoagulant activity. Activated protein C inactivates thrombin-activated FVIII through cleavage adjacent to position ...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00125a022

    authors: O'Brien DP,Johnson D,Byfield P,Tuddenham EG

    更新日期:1992-03-17 00:00:00

  • Ethylene enhances reactivity of superoxide with peroxidase to form the oxy-ferrous complex.

    abstract::Ethylene and its analogues acetylene, carbon monoxide, and propylene inhibited the rate of oxidation of indole-3-acetic acid by peroxidase. Annulment of this effect by addition of superoxide dismutase showed that inhibition occurred only in the presence of the superoxide anion radical (O2-.). Kinetic and spectral data...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00276a019

    authors: Smith AM,Morrison WL,Milham PJ

    更新日期:1983-03-29 00:00:00

  • Prion-like p53 Amyloids in Cancer.

    abstract::The global transcription factor, p53, is a master regulator of gene expression in cells. Mutations in the TP53 gene promote unregulated cell growth through the inactivation of downstream effectors of the p53 pathway. In fact, mutant p53 is highly prone to misfolding and frequently resides inside the cell as large aggr...

    journal_title:Biochemistry

    pub_type: 杂志文章,评审

    doi:10.1021/acs.biochem.9b00796

    authors: Navalkar A,Ghosh S,Pandey S,Paul A,Datta D,Maji SK

    更新日期:2020-01-21 00:00:00

  • Structural, dynamic, and chemical characterization of a novel S-glycosylated bacteriocin.

    abstract::Bacteriocins are bacterial peptides with specific activity against competing species. They hold great potential as natural preservatives and for their probiotic effects. We show here nuclear magnetic resonance-based evidence that glycocin F, a 43-amino acid bacteriocin from Lactobacillus plantarum, contains two β-link...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi200217u

    authors: Venugopal H,Edwards PJ,Schwalbe M,Claridge JK,Libich DS,Stepper J,Loo T,Patchett ML,Norris GE,Pascal SM

    更新日期:2011-04-12 00:00:00

  • 2D NMR studies and 3D structure of the parallel-stranded duplex oligonucleotide Acrm5-alpha-d(TCTAAACTC)-beta-d(AGATTTGAG) via complete relaxation matrix analysis of the NOE effects and molecular mechanics calculations.

    abstract::The three-dimensional structure of the duplex formed by the association of the unnatural oligonucleotide alpha-d(TCTAAACTC) covalently linked to an acridine derivative (m5Acr) with its natural and parallel complementary sequence beta-d(AGATTTGAG) was investigated by nuclear magnetic resonance spectroscopy and constrai...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00472a031

    authors: Guesnet JL,Vovelle F,Thuong NT,Lancelot G

    更新日期:1990-05-22 00:00:00

  • Inhibition of human leukocyte elastase, cathepsin G, chymotrypsin A alpha, and porcine pancreatic elastase with substituted isobenzofuranones and benzopyrandiones.

    abstract::Several 3-halo-3-(1-haloalkyl)-1(3H)-isobenzofuranones, 3-(1-haloalkylidene)-1(3H)-isobenzofuranones, and 3-bromomethyl-1H-2-benzopyran-1-ones containing masked halo ketone functional groups were synthesized and tested as inhibitors of several serine proteases including human leukocyte (HL) elastase and cathepsin G. W...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00329a006

    authors: Hemmi K,Harper JW,Powers JC

    更新日期:1985-04-09 00:00:00

  • Molecular structure of Escherichia coli PurT-encoded glycinamide ribonucleotide transformylase.

    abstract::In Escherichia coli, the PurT-encoded glycinamide ribonucleotide transformylase, or PurT transformylase, catalyzes an alternative formylation of glycinamide ribonucleotide (GAR) in the de novo pathway for purine biosynthesis. On the basis of amino acid sequence analyses, it is known that the PurT transformylase belong...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi000926j

    authors: Thoden JB,Firestine S,Nixon A,Benkovic SJ,Holden HM

    更新日期:2000-08-01 00:00:00

  • Inhibition of protein interactions with the beta 2 sliding clamp of Escherichia coli DNA polymerase III by peptides from beta 2-binding proteins.

    abstract::The sliding clamp of the Escherichia coli replisome is now understood to interact with many proteins involved in DNA synthesis and repair. A universal interaction motif is proposed to be one mechanism by which those proteins bind the E. coli sliding clamp, a homodimer of the beta subunit, at a single site on the dimer...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi036229j

    authors: Wijffels G,Dalrymple BP,Prosselkov P,Kongsuwan K,Epa VC,Lilley PE,Jergic S,Buchardt J,Brown SE,Alewood PF,Jennings PA,Dixon NE

    更新日期:2004-05-18 00:00:00

  • Unique Conformation in a Natural Interruption Sequence of Type XIX Collagen Revealed by Its High-Resolution Crystal Structure.

    abstract::Naturally occurring interruptions in nonfibrillar collagen play key roles in molecular flexibility, collagen degradation, and ligand binding. The structural feature of the interruption sequences and the molecular basis for their functions have not been well studied. Here, we focused on a G5G type natural interruption ...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/acs.biochem.7b01010

    authors: Xu T,Zhou CZ,Xiao J,Liu J

    更新日期:2018-02-20 00:00:00

  • Structural basis for substrate binding and the catalytic mechanism of type III pantothenate kinase.

    abstract::Pantothenate kinase (PanK) catalyzes the first step of the universal five-step coenzyme A (CoA) biosynthetic pathway. The recently characterized type III PanK (PanK-III, encoded by the coaX gene) is distinct in sequence, structure and enzymatic properties from both the long-known bacterial type I PanK (PanK-I, exempli...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi7018578

    authors: Yang K,Strauss E,Huerta C,Zhang H

    更新日期:2008-02-05 00:00:00

  • Comparative studies on the 7-iodo and 7-fluoro derivatives of N-acetoxy-N-2-acetylaminofluorene: binding sites on DNA and conformational change of modified deoxytrinucleotides.

    abstract::GMP and native DNA were reacted with 7-iodo and 7-fluoro derivatives of N-acetoxy-N-2-acetylaminofluorene. It was shown that the 7-halogeno derivatives react on C-8 of guanine. Furthermore the respective amount of arylamidation (covalent linkage on the C-8 of guanine) and arylation (covalent linkage on 2-NH2 groups of...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00606a016

    authors: Lefèvre JF,Fuchs RP,Daune MP

    更新日期:1978-06-27 00:00:00

  • Long-range interaction networks in the function and fidelity of poliovirus RNA-dependent RNA polymerase studied by nuclear magnetic resonance.

    abstract::The fidelity of the poliovirus RNA-dependent RNA polymerase (3D(pol)) plays a direct role in the genomic evolution and pathogenesis of the virus. A single site mutation (Gly64Ser) that is remote from the catalytic center results in a higher fidelity polymerase. NMR studies with [methyl-(13)C]methionine-labeled protein...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi100833r

    authors: Yang X,Welch JL,Arnold JJ,Boehr DD

    更新日期:2010-11-02 00:00:00

  • Chemical tools for K(+) channel biology.

    abstract::K(+) channels are revered for their universal action of suppressing electrical activity in nerve and muscle, as well as regulating salt and water transport in epithelial tissues involved in metabolism and digestion. These multisubunit membrane-embedded proteins carry out their physiological chore, selectively allowing...

    journal_title:Biochemistry

    pub_type: 杂志文章,评审

    doi:10.1021/bi8018515

    authors: Ahern CA,Kobertz WR

    更新日期:2009-01-27 00:00:00

  • Reactivity of sulfenic acid in human serum albumin.

    abstract::Sulfenic acid is formed upon oxidation of thiols and is a central intermediate in the redox modulation of an increasing number of proteins. Methods for quantifying or even detecting sulfenic acid are scarce. Herein, the reagent 7-chloro-4-nitrobenz-2-oxa-1,3-diazole was determined not to be suitable as a chromophoric ...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi701520y

    authors: Turell L,Botti H,Carballal S,Ferrer-Sueta G,Souza JM,Durán R,Freeman BA,Radi R,Alvarez B

    更新日期:2008-01-08 00:00:00

  • Inactivation of C30A trimethylamine dehydrogenase by N-cyclopropyl-alpha-methylbenzylamine, 1-phenylcyclopropylamine, and phenylhydrazine.

    abstract::Trimethylamine dehydrogenase (TMADH) from the bacterium Methylophilus methylotrophus (sp. W(3)A(1)) and its C30A mutant were inactivated with three known inactivators of monoamine oxidase, namely, phenylhydrazine, N-cyclopropyl-alpha-methylbenzylamine, and 1-phenylcyclopropylamine. All three compounds irreversibly ina...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi0105126

    authors: Mitchell DJ,Nikolic D,Jang MH,van Breemen RB,Hille R,Silverman RB

    更新日期:2001-07-24 00:00:00

  • Tandem GA residues on opposite sides of the loop in molecular beacon-like DNA hairpins compact the loop and increase hairpin stability.

    abstract::The free solution electrophoretic mobilities and thermal stabilities of hairpins formed by two complementary 26-nucleotide oligomers have been measured by capillary electrophoresis. The oligomers are predicted to form molecular beacon-like hairpins with 5 bp stems and 16 nucleotides in the loop. One hairpin, called ha...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi201263n

    authors: Chang CY,Stellwagen NC

    更新日期:2011-10-25 00:00:00

  • Phosphoprotein particles: calcium and inorganic phosphate binding structures.

    abstract::Phosphoprotein particles were isolated in their native state from the physiological fluid of the estuarine clam Rangia cuneata , and the characteristics of the mineral ion-protein complex which constitutes the native particle were investigated by using mineral ion binding and mineral ion exchange techniques. The parti...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00302a017

    authors: Marsh ME,Sass RL

    更新日期:1984-03-27 00:00:00

  • Evidence of random structural features in the heparin polymer.

    abstract::The first use of computer-simulation studies to examine heparin's structure has been reported. The product distributions obtained when porcine mucosal heparins were depolymerized with heparinase have been compared to computer-simulated distributions. The modeled distribution was relatively unaffected by the polydisper...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi00347a045

    authors: Linhardt RJ,Merchant ZM,Rice KG,Kim YS,Fitzgerald GL,Grant AC,Langer R

    更新日期:1985-12-17 00:00:00

  • Functional epitopes for site 1 of human prolactin.

    abstract::Human prolactin (hPRL) binds two human prolactin receptor molecules, creating active heterotrimeric complexes. Receptors bind dissimilar hormone surfaces termed site 1 and site 2 in an obligate ordered process. We sought to map the functional epitopes in site 1 of hPRL. Extensive alanine mutagenesis (102 of the 199 re...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi101838s

    authors: Rao GV,Brooks CL

    更新日期:2011-03-01 00:00:00

  • An infrared spectroscopic study of the secondary structure of protein kinase C alpha and its thermal denaturation.

    abstract::The secondary structure of protein kinase C alpha (PKC alpha) has been studied using infrared spectroscopy in the presence of both H(2)O and D(2)O buffers. In the absence of ligands at 20 degrees C, it was shown that beta-sheet is the major component, representing about 44% of the total structure, whereas the alpha-he...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi035128i

    authors: Torrecillas A,Corbalán-García S,Gómez-Fernández JC

    更新日期:2004-03-02 00:00:00

  • Phosphoprotein and phosphopeptide interactions with the FHA domain from Arabidopsis kinase-associated protein phosphatase.

    abstract::FHA domains are phosphoThr recognition modules found in diverse signaling proteins, including kinase-associated protein phosphatase (KAPP) from Arabidopsis thaliana. The kinase-interacting FHA domain (KI-FHA) of KAPP targets it to function as a negative regulator of some receptor-like kinase (RLK) signaling pathways i...

    journal_title:Biochemistry

    pub_type: 杂志文章

    doi:10.1021/bi061763n

    authors: Ding Z,Wang H,Liang X,Morris ER,Gallazzi F,Pandit S,Skolnick J,Walker JC,Van Doren SR

    更新日期:2007-03-13 00:00:00