CSA-dependent degradation of CSB by the ubiquitin-proteasome pathway establishes a link between complementation factors of the Cockayne syndrome.


:Mutations in the CSA or CSB complementation genes cause the Cockayne syndrome, a severe genetic disorder that results in patients' death in early adulthood. CSA and CSB act in a transcription-coupled repair (TCR) pathway, but their functional relationship is not understood. We have previously shown that CSA is a subunit of an E3 ubiquitin ligase complex. Here we demonstrate that CSB is a substrate of this ligase: Following UV irradiation, CSB is degraded at a late stage of the repair process in a proteasome- and CSA-dependent manner. Moreover, we demonstrate the importance of CSB degradation for post-TCR recovery of transcription and for the Cockayne syndrome. Our results unravel for the first time the functional relationship between CSA and CSB.


Genes Dev


Genes & development


Groisman R,Kuraoka I,Chevallier O,Gaye N,Magnaldo T,Tanaka K,Kisselev AF,Harel-Bellan A,Nakatani Y




Has Abstract


2006-06-01 00:00:00














  • Desert Hedgehog/Patched 1 signaling specifies fetal Leydig cell fate in testis organogenesis.

    abstract::Establishment of the steroid-producing Leydig cell lineage is an event downstream of Sry that is critical for masculinization of mammalian embryos. Neither the origin of fetal Leydig cell precursors nor the signaling pathway that specifies the Leydig cell lineage is known. Based on the sex-specific expression patterns...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Yao HH,Whoriskey W,Capel B

    更新日期:2002-06-01 00:00:00

  • Control of a neuronal morphology program by an RNA-binding zinc finger protein, Unkempt.

    abstract::Cellular morphology is an essential determinant of cellular function in all kingdoms of life, yet little is known about how cell shape is controlled. Here we describe a molecular program that controls the early morphology of neurons through a metazoan-specific zinc finger protein, Unkempt. Depletion of Unkempt in mous...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Murn J,Zarnack K,Yang YJ,Durak O,Murphy EA,Cheloufi S,Gonzalez DM,Teplova M,Curk T,Zuber J,Patel DJ,Ule J,Luscombe NM,Tsai LH,Walsh CA,Shi Y

    更新日期:2015-03-01 00:00:00

  • Dynamic changes in histone modifications precede de novo DNA methylation in oocytes.

    abstract::Erasure and subsequent reinstatement of DNA methylation in the germline, especially at imprinted CpG islands (CGIs), is crucial to embryogenesis in mammals. The mechanisms underlying DNA methylation establishment remain poorly understood, but a number of post-translational modifications of histones are implicated in a...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Stewart KR,Veselovska L,Kim J,Huang J,Saadeh H,Tomizawa S,Smallwood SA,Chen T,Kelsey G

    更新日期:2015-12-01 00:00:00

  • Pit-1 binding to specific DNA sites as a monomer or dimer determines gene-specific use of a tyrosine-dependent synergy domain.

    abstract::Transcriptional activation of the prolactin and growth hormone genes, occurring in a cell-specific fashion, requires short-range synergistic interactions between the pituitary-specific POU domain factor Pit-1 and other transcription factors, particularly nuclear receptors. Unexpectedly, we find that these events invol...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Holloway JM,Szeto DP,Scully KM,Glass CK,Rosenfeld MG

    更新日期:1995-08-15 00:00:00

  • Inhibition of viral gene expression by the catalytic RNA subunit of RNase P from Escherichia coli.

    abstract::The catalytic RNA subunit (M1 RNA) of RNase P from Escherichia coli has been converted to an endoribonuclease that specifically cleaves the mRNA that encodes thymidine kinase (TK) of herpes simplex virus 1 (HSV-1). Covalent attachment to the 3' end of M1 RNA of a sequence complementary to TK mRNA results in very effic...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Liu F,Altman S

    更新日期:1995-02-15 00:00:00

  • Ectodermal Wnt3/beta-catenin signaling is required for the establishment and maintenance of the apical ectodermal ridge.

    abstract::The formation of the apical ectodermal ridge (AER) is critical for the distal outgrowth and patterning of the vertebrate limb. Recent work in the chick has demonstrated that interplay between the Wnt and Fgf signaling pathways is essential in the limb mesenchyme and ectoderm in the establishment and perhaps the mainte...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Barrow JR,Thomas KR,Boussadia-Zahui O,Moore R,Kemler R,Capecchi MR,McMahon AP

    更新日期:2003-02-01 00:00:00

  • USP21 deubiquitinase promotes pancreas cancer cell stemness via Wnt pathway activation.

    abstract::The ubiquitin-specific protease (USP) family is the largest group of cysteine proteases. Cancer genomic analysis identified frequent amplification of USP21 (22%) in human pancreatic ductal adenocarcinoma (PDAC). USP21 overexpression correlates with human PDAC progression, and enforced expression of USP21 accelerates m...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Hou P,Ma X,Zhang Q,Wu CJ,Liao W,Li J,Wang H,Zhao J,Zhou X,Guan C,Ackroyd J,Jiang S,Zhang J,Spring DJ,Wang YA,DePinho RA

    更新日期:2019-10-01 00:00:00

  • Null mutation of the prolactin receptor gene produces multiple reproductive defects in the mouse.

    abstract::Mice carrying a germ-line null mutation of the prolactin receptor gene have been produced by gene targeting in embryonic stem cells. Heterozygous females showed almost complete failure of lactation attributable to greatly reduced mammary gland development after their first, but not subsequent, pregnancies. Homozygous ...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Ormandy CJ,Camus A,Barra J,Damotte D,Lucas B,Buteau H,Edery M,Brousse N,Babinet C,Binart N,Kelly PA

    更新日期:1997-01-15 00:00:00

  • c-Jun is required for the specification of joint cell fates.

    abstract::Joints form within the developing skeleton through the segmentation and cavitation of initially continuous cartilage condensations. However, the molecular pathways controlling joint formation largely remain to be clarified. In particular, while several critical secreted signals have been identified, no transcription f...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Kan A,Tabin CJ

    更新日期:2013-03-01 00:00:00

  • Analysis of the lethal interaction between the prune and Killer of prune mutations of Drosophila.

    abstract::The third-chromosome mutation Killer of prune (K-pn) causes no phenotype by itself, but causes lethality in individuals homozygous for the nonlethal X-chromosome mutation prune (pn). We have recovered 12 gamma-ray-induced revertants of Killer of prune. All of the revertants fail to complement a recessive cell lethal m...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Biggs J,Tripoulas N,Hersperger E,Dearolf C,Shearn A

    更新日期:1988-10-01 00:00:00

  • Purification of the human histone H4 gene-specific transcription factors H4TF-1 and H4TF-2.

    abstract::Transcription of the cell-cycle-regulated human histone genes increases approximately fivefold during S phase. One step toward the elucidation of the biochemical mechanisms that govern cell-cycle-regulated expression of these genes is to purify and characterize the transcription factors that regulate these promoters. ...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Dailey L,Roberts SB,Heintz N

    更新日期:1988-12-01 00:00:00

  • G1 cyclin-dependent kinases are insufficient to reverse dE2F2-mediated repression.

    abstract::Here we show that the cell cycle defects of dE2F1-depleted cells depend on the cooperative effects of dE2F2 and DACAPO (DAP), an inhibitor of Cyclin E/cyclin-dependent kinase 2 (CycE/cdk2). The different properties of cells lacking dE2F1/dE2F2 and dE2F1/DAP lead to the surprising observation that dE2F2-mediated repres...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Frolov MV,Stevaux O,Moon NS,Dimova D,Kwon EJ,Morris EJ,Dyson NJ

    更新日期:2003-03-15 00:00:00

  • FILAMENTOUS FLOWER, a meristem and organ identity gene of Arabidopsis, encodes a protein with a zinc finger and HMG-related domains.

    abstract::Distinctive from that of the animal system, the basic plan of the plant body is the continuous formation of a structural unit, composed of a stem with a meristem at the top and lateral organs continuously forming at the meristem. Therefore, mechanisms controlling the formation, maintenance, and development of a merist...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Sawa S,Watanabe K,Goto K,Liu YG,Shibata D,Kanaya E,Morita EH,Okada K

    更新日期:1999-05-01 00:00:00

  • Fragile X-related protein and VIG associate with the RNA interference machinery.

    abstract::RNA interference (RNAi) is a flexible gene silencing mechanism that responds to double-stranded RNA by suppressing homologous genes. Here, we report the characterization of RNAi effector complexes (RISCs) that contain small interfering RNAs and microRNAs (miRNAs). We identify two putative RNA-binding proteins, the Dro...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Caudy AA,Myers M,Hannon GJ,Hammond SM

    更新日期:2002-10-01 00:00:00

  • Four histone variants mark the boundaries of polycistronic transcription units in Trypanosoma brucei.

    abstract::Unusually for a eukaryote, genes transcribed by RNA polymerase II (pol II) in Trypanosoma brucei are arranged in polycistronic transcription units. With one exception, no pol II promoter motifs have been identified, and how transcription is initiated remains an enigma. T. brucei has four histone variants: H2AZ, H2BV, ...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Siegel TN,Hekstra DR,Kemp LE,Figueiredo LM,Lowell JE,Fenyo D,Wang X,Dewell S,Cross GA

    更新日期:2009-05-01 00:00:00

  • Changes in histone gene dosage alter transcription in yeast.

    abstract::Chromatin structure is believed to be important for a number of cellular processes, including transcription. However, the role of nucleosomes in transcription is not well understood. We have identified the yeast histone locus HTB1-HTB1, encoding histones H2A and H2B, as a suppressor of solo delta insertion mutations t...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Clark-Adams CD,Norris D,Osley MA,Fassler JS,Winston F

    更新日期:1988-02-01 00:00:00

  • Genetic and biochemical definition of the Hedgehog receptor.

    abstract::Although the transporter-like protein Patched (Ptc) is genetically implicated in reception of the extracellular Hedgehog (Hh) protein signal, a clear definition of the Hh receptor is complicated by the existence of additional Hh-binding proteins and, in Drosophila, by the lack of physical evidence for direct binding o...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Zheng X,Mann RK,Sever N,Beachy PA

    更新日期:2010-01-01 00:00:00

  • SWI/SNF regulates a transcriptional program that induces senescence to prevent liver cancer.

    abstract::Oncogene-induced senescence (OIS) is a potent tumor suppressor mechanism. To identify senescence regulators relevant to cancer, we screened an shRNA library targeting genes deleted in hepatocellular carcinoma (HCC). Here, we describe how knockdown of the SWI/SNF component ARID1B prevents OIS and cooperates with RAS to...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Tordella L,Khan S,Hohmeyer A,Banito A,Klotz S,Raguz S,Martin N,Dhamarlingam G,Carroll T,González Meljem JM,Deswal S,Martínez-Barbera JP,García-Escudero R,Zuber J,Zender L,Gil J

    更新日期:2016-10-01 00:00:00

  • Transcription factor CREM coordinates the timing of hepatocyte proliferation in the regenerating liver.

    abstract::The liver regenerates upon partial hepatectomy (PH) as terminally differentiated hepatocytes undergo a tremendous proliferative process. CREM gene expression is powerfully induced during liver regeneration. We show that cell proliferation is significantly reduced upon PH in CREM-/- mice. There is a reduction in DNA sy...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Servillo G,Della Fazia MA,Sassone-Corsi P

    更新日期:1998-12-01 00:00:00

  • The C. elegans microRNA let-7 binds to imperfect let-7 complementary sites from the lin-41 3'UTR.

    abstract::Caenorhabditis elegans let-7, a founding member of the microRNA family, is predicted to bind to six sites in the 3'UTR of the mRNA of its target gene, lin-41, to down-regulate LIN-41. Here, we demonstrate that wild-type let-7 microRNA binds in vitro to RNA from the lin-41 3'UTR. This interaction is dependent on two co...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Vella MC,Choi EY,Lin SY,Reinert K,Slack FJ

    更新日期:2004-01-15 00:00:00

  • Sex-specific regulation of the male-specific lethal-1 dosage compensation gene in Drosophila.

    abstract::Dosage compensation in Drosophila occurs by a twofold increase in transcription per copy of X-linked genes in males (XY) compared with females (XX). msl-1 is one of four genes that are essential for dosage compensation in males, and MSL-1 protein is associated specifically with the male X chromosome. To explore the ba...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Palmer MJ,Richman R,Richter L,Kuroda MI

    更新日期:1994-03-15 00:00:00

  • On the role of homologous sequences in chromosomal rearrangements.

    abstract::Deletions and other chromosomal rearrangements can be generated by recombination between repeated sequences. It has been shown in a number of systems that the probability of exchange or gene conversion decreases with increasing distance between repeats. This paper examines the question of how repeats find each other, ...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Singer BS

    更新日期:1988-12-01 00:00:00

  • Nascent peptide-mediated translation elongation arrest coupled with mRNA degradation in the CGS1 gene of Arabidopsis.

    abstract::Expression of the Arabidopsis CGS1 gene that codes for cystathionine gamma-synthase is feedback regulated at the step of mRNA stability in response to S-adenosyl-L-methionine (AdoMet). A short stretch of amino acid sequence, called the MTO1 region, encoded by the first exon of CGS1 itself is involved in this regulatio...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Onouchi H,Nagami Y,Haraguchi Y,Nakamoto M,Nishimura Y,Sakurai R,Nagao N,Kawasaki D,Kadokura Y,Naito S

    更新日期:2005-08-01 00:00:00

  • Analysis of a noncanonical poly(A) site reveals a tripartite mechanism for vertebrate poly(A) site recognition.

    abstract::At least half of all human pre-mRNAs are subject to alternative 3' processing that may modulate both the coding capacity of the message and the array of post-transcriptional regulatory elements embedded within the 3' UTR. Vertebrate poly(A) site selection appears to rely primarily on the binding of CPSF to an A(A/U)UA...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Venkataraman K,Brown KM,Gilmartin GM

    更新日期:2005-06-01 00:00:00

  • Caenorhabditis elegans sex-determining protein FEM-2 is a protein phosphatase that promotes male development and interacts directly with FEM-3.

    abstract::Male sexual development in the nematode Caenorhabditis elegans requires the genes fem-1, fem-2, and fem-3. The current model of sex determination portrays the FEM proteins as components of a novel signal transduction pathway, but the mechanisms involved in signaling through the pathway are not understood. We report th...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Chin-Sang ID,Spence AM

    更新日期:1996-09-15 00:00:00

  • scratch, a pan-neural gene encoding a zinc finger protein related to snail, promotes neuronal development.

    abstract::The Drosophila scratch (scrt) gene is expressed in most or all neuronal precursor cells and encodes a predicted zinc finger transcription factor closely related to the product of the mesoderm determination gene snail (sna). Adult flies homozygous for scrt null alleles have a reduced number of photoreceptors in the eye...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Roark M,Sturtevant MA,Emery J,Vaessin H,Grell E,Bier E

    更新日期:1995-10-01 00:00:00

  • Negative regulation of FAR1 at the Start of the yeast cell cycle.

    abstract::In budding yeast, a switch between the mutually exclusive pathways of cell cycle progression and conjugation is controlled at Start in late G1 phase. Mating pheromones promote conjugation by arresting cells in G1 phase before Start. Pheromone-induced cell cycle arrest requires a functional FAR1 gene. We have found tha...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: McKinney JD,Chang F,Heintz N,Cross FR

    更新日期:1993-05-01 00:00:00

  • Opposing effects of TIGAR- and RAC1-derived ROS on Wnt-driven proliferation in the mouse intestine.

    abstract::Reactive oxygen species (ROS) participate in numerous cell responses, including proliferation, DNA damage, and cell death. Based on these disparate activities, both promotion and inhibition of ROS have been proposed for cancer therapy. However, how the ROS response is determined is not clear. We examined the activitie...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Cheung EC,Lee P,Ceteci F,Nixon C,Blyth K,Sansom OJ,Vousden KH

    更新日期:2016-01-01 00:00:00

  • Spatial arrangement of an RNA zipcode identifies mRNAs under post-transcriptional control.

    abstract::How RNA-binding proteins recognize specific sets of target mRNAs remains poorly understood because current approaches depend primarily on sequence information. In this study, we demonstrate that specific recognition of messenger RNAs (mRNAs) by RNA-binding proteins requires the correct spatial positioning of these seq...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Patel VL,Mitra S,Harris R,Buxbaum AR,Lionnet T,Brenowitz M,Girvin M,Levy M,Almo SC,Singer RH,Chao JA

    更新日期:2012-01-01 00:00:00

  • Half helical turn spacing changes convert a frog into a mouse rDNA promoter: a distant upstream domain determines the helix face of the initiation site.

    abstract::Transcription of frog rDNA by mouse cell factors is the only documented exception to the observed species selectivity of rRNA gene expression. This heterologous transcription is authentic in that it uses the normal frog upstream and core promoter domains, as well as the normal mouse polymerase I transcription factors,...

    journal_title:Genes & development

    pub_type: 杂志文章


    authors: Pape LK,Windle JJ,Sollner-Webb B

    更新日期:1990-01-01 00:00:00