Abstract:
:Primary cilium dysfunction underlies the pathogenesis of Bardet-Biedl syndrome (BBS), a genetic disorder whose symptoms include obesity, retinal degeneration, and nephropathy. However, despite the identification of 12 BBS genes, the molecular basis of BBS remains elusive. Here we identify a complex composed of seven highly conserved BBS proteins. This complex, the BBSome, localizes to nonmembranous centriolar satellites in the cytoplasm but also to the membrane of the cilium. Interestingly, the BBSome is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Strikingly, Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Conversely, preventing Rab8(GTP) production blocks ciliation in cells and yields characteristic BBS phenotypes in zebrafish. Our data reveal that BBS may be caused by defects in vesicular transport to the cilium.
journal_name
Celljournal_title
Cellauthors
Nachury MV,Loktev AV,Zhang Q,Westlake CJ,Peränen J,Merdes A,Slusarski DC,Scheller RH,Bazan JF,Sheffield VC,Jackson PKdoi
10.1016/j.cell.2007.03.053subject
Has Abstractpub_date
2007-06-15 00:00:00pages
1201-13issue
6eissn
0092-8674issn
1097-4172pii
S0092-8674(07)00534-Xjournal_volume
129pub_type
杂志文章相关文献
CELL文献大全abstract::In yeast, hyperosmotic stress causes an immediate dissociation of most proteins from chromatin, presumably because cells are unprepared for, and initially unresponsive to, increased ion concentrations in the nucleus. Osmotic stress activates Hog1 MAP kinase, which phosphorylates at least two proteins located at the pl...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2004.07.016
更新日期:2004-08-06 00:00:00
abstract::Following EMS mutagenesis we recovered a mutant of D. melanogaster that grows at concentrations of alpha-amanitin lethal to wild-type. To our knowledge this mutant represents the first example of an amanitin-resistant eucaryotic organism. The amanitin resistance of the mutant (AmaC4) is due to an alteration in its DNA...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(79)90116-8
更新日期:1979-11-01 00:00:00
abstract::Constitutive cell-autonomous immunity in metazoans predates interferon-inducible immunity and comprises primordial innate defense. Phagocytes mobilize interferon-inducible responses upon engagement of well-characterized signaling pathways by pathogen-associated molecular patterns (PAMPs). The signals controlling deplo...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2017.09.034
更新日期:2017-11-02 00:00:00
abstract::Cancer cell of origin is difficult to identify by analyzing cells within terminal stage tumors, whose identity could be concealed by the acquired plasticity. Thus, an ideal approach to identify the cell of origin is to analyze proliferative abnormalities in distinct lineages prior to malignancy. Here, we use mosaic an...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2011.06.014
更新日期:2011-07-22 00:00:00
abstract::Gene silencing is instrumental to interrogate gene function and holds promise for therapeutic applications. Here, we repurpose the endogenous retroviruses' silencing machinery of embryonic stem cells to stably silence three highly expressed genes in somatic cells by epigenetics. This was achieved by transiently expres...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2016.09.006
更新日期:2016-09-22 00:00:00
abstract::The dual host range virus HIX has been previously characterized as an envelope gene recombinant between Moloney murine leukemia virus (Mo-MuLV) and an unidentified xenotropic murine leukemia virus. Using long reverse transcripts of Mo-MuLV, a region of nonhomology has been mapped by electron microscopic analysis of he...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(78)90350-1
更新日期:1978-08-01 00:00:00
abstract::The budding yeast, Saccharomyces cerevisiae, has emerged as an archetype of eukaryotic cell biology. Here we show that S. cerevisiae is also a model for the evolution of cooperative behavior by revisiting flocculation, a self-adherence phenotype lacking in most laboratory strains. Expression of the gene FLO1 in the la...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2008.09.037
更新日期:2008-11-14 00:00:00
abstract::Transcription hinders replication fork progression and stability. The ATR checkpoint and specialized DNA helicases assist DNA synthesis across transcription units to protect genome integrity. Combining genomic and genetic approaches together with the analysis of replication intermediates, we searched for factors coord...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2012.09.041
更新日期:2012-11-09 00:00:00
abstract::During the postpartum period, the brain's inhibitory GABAA receptors may not recover in time following their reduced numbers during pregnancy. This is likely the cause of postpartum depression prevalent in ∼12% of childbearing women. A new therapy for this condition consists of administering a synthetic neurosteroid d...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2018.12.016
更新日期:2019-01-10 00:00:00
abstract::Understanding how transcriptional enhancers control over 20,000 protein-coding genes to maintain cell-type-specific gene expression programs in all human cells is a fundamental challenge in regulatory biology. Recent studies suggest that gene regulatory elements and their target genes generally occur within insulated ...
journal_title:Cell
pub_type: 杂志文章,评审
doi:10.1016/j.cell.2016.10.024
更新日期:2016-11-17 00:00:00
abstract::Immune responses involve coordination across cell types and tissues. However, studies in cancer immunotherapy have focused heavily on local immune responses in the tumor microenvironment. To investigate immune activity more broadly, we performed an organism-wide study in genetically engineered cancer models using mass...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2016.12.022
更新日期:2017-01-26 00:00:00
abstract::Histone methylation regulates chromatin structure and transcription. The recently identified histone demethylase lysine-specific demethylase 1 (LSD1) is chemically restricted to demethylation of only mono- and di- but not trimethylated histone H3 lysine 4 (H3K4me3). We show that the X-linked mental retardation (XLMR) ...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2007.02.017
更新日期:2007-03-23 00:00:00
abstract::The mechanism by which nucleosome cores are displaced and re-formed during transcription in vitro has been investigated. A nucleosome core was assembled on a short linear DNA template (227 bp) containing an SP6 RNA polymerase promoter and a nucleosome-positioning sequence. Transcription induced the translocation of th...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(94)90343-3
更新日期:1994-01-28 00:00:00
abstract::Four major receptor families enable cells to respond to chemical and physical signals from their proximal environment. The ligand- and voltage-gated ion channels, G-protein-coupled receptors, nuclear hormone receptors, and receptor tyrosine kinases are all allosteric proteins that carry multiple, spatially distinct, y...
journal_title:Cell
pub_type: 杂志文章,评审
doi:10.1016/j.cell.2016.08.015
更新日期:2016-08-25 00:00:00
abstract::At the replication origin of ColE1 plasmid, a persistent hybrid is formed between the primer precursor (RNA II) and its template DNA. The wild-type sequence in the region 13 to 20 bp upstream (-20 region) of the origin is required to form this persistent hybrid. While the template strand for transcription of this regi...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(90)90370-t
更新日期:1990-07-27 00:00:00
abstract::The proportion of BFU-E normally engaged in DNA synthesis is low in adult B6 (C57BL/6) mice of genotype Fv2rr (resistant to Friend erythroleukemia virus), as shown by 3H-thymidine or hydroxyurea "cell suicide" experiments in vivo and vitro. When bone marrow cells from these mice were subjected to a single wash in alph...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(81)90306-8
更新日期:1981-10-01 00:00:00
abstract::DNA double-strand breaks (DSBs) in B lymphocytes arise stochastically during replication or as a result of targeted DNA damage by activation-induced cytidine deaminase (AID). Here we identify recurrent, early replicating, and AID-independent DNA lesions, termed early replication fragile sites (ERFSs), by genome-wide l...
journal_title:Cell
pub_type: 杂志文章,评审
doi:10.1016/j.cell.2013.01.006
更新日期:2013-01-31 00:00:00
abstract::The Hippo-YAP pathway regulates organ size by modulating cell proliferation and apoptosis. Yu et al. now reveal that G-protein-coupled receptors act upstream of the transcriptional coactivators YAP/TAZ. This study reinforces the connection between the actin cytoskeleton and Hippo pathway activity and identifies a clas...
journal_title:Cell
pub_type: 评论,杂志文章
doi:10.1016/j.cell.2012.07.020
更新日期:2012-08-17 00:00:00
abstract::Microglia are the resident macrophages of the CNS, and their functions have been extensively studied in various brain pathologies. The physiological roles of microglia in brain plasticity and function, however, remain unclear. To address this question, we generated CX3CR1(CreER) mice expressing tamoxifen-inducible Cre...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2013.11.030
更新日期:2013-12-19 00:00:00
abstract::Poorly immunogenic tumor cells evade host immunity and grow even in the presence of an intact immune system, but the complex mechanisms regulating tumor immunogenicity have not been elucidated. Here, we discovered an unexpected role of the Hippo pathway in suppressing anti-tumor immunity. We demonstrate that, in three...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2016.11.005
更新日期:2016-12-01 00:00:00
abstract::The messenger RNA coding for the precursor of the small subunit of ribulose--1,5--bisphosphate (Ru-P2) carboxylase has been partially purified from pea leaves. The mRNA has a size of 11.5S, is approximately 800 nucleotides long and, on cell-free translation, directs the synthesis of a single major polypeptide of 20,00...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(79)90164-8
更新日期:1979-06-01 00:00:00
abstract::The cpd mutation localized by T-DNA tagging on Arabidopsis chromosome 5-14.3 inhibits cell elongation controlled by the ecdysone-like brassinosteroid hormone brassinolide. The cpd mutant displays de-etiolation and derepression of light-induced genes in the dark, as well as dwarfism, male sterility, and activation of s...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/s0092-8674(00)81094-6
更新日期:1996-04-19 00:00:00
abstract::During eukaryotic translation initiation, initiator tRNA does not insert fully into the P decoding site on the 40S ribosomal subunit. This conformation (POUT) is compatible with scanning mRNA for the AUG start codon. Base pairing with AUG is thought to promote isomerization to a more stable conformation (PIN) that arr...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/j.cell.2014.10.001
更新日期:2014-10-23 00:00:00
abstract::X-ray studies have established that the structure of a right-handed, Watson-Crick double helix can change from place to place along its length as a function of base sequence. The base pairs transmit deformations out to the phosphate backbone, where they can then be recognized by proteins and other DNA-binding reagents...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(84)90379-9
更新日期:1984-06-01 00:00:00
abstract::Cyclic dinucleotides (CDNs) have been previously recognized as important secondary signaling molecules in bacteria and, more recently, in mammalian cells. In the former case, they represent secondary messengers affecting numerous responses of the prokaryotic cell, whereas in the latter, they act as agonists of the inn...
journal_title:Cell
pub_type: 杂志文章,评审
doi:10.1016/j.cell.2013.08.014
更新日期:2013-08-29 00:00:00
abstract::Monkey cells synthesizing SV40 large T antigen were lysed and the extracts immunoprecipitated with either monoclonal anti-T antibody or monoclonal antibody to p110-114, the product of the retinoblastoma susceptibility gene (Rb). T and p110-114 coprecipitated in each case, implying that the proteins are complexed with ...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(88)90559-4
更新日期:1988-07-15 00:00:00
abstract::The asymmetries of internal organs are consistently oriented along the left-right axis in all vertebrates, and perturbations of left-right orientation lead to significant congenital disease. We propose a model in which a "left-right coordinator" interacts with the Spemann organizer to coordinate the evolutionarily con...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/s0092-8674(00)81144-7
更新日期:1998-04-03 00:00:00
abstract::We have isolated a population of vesicular carriers involved in the transport (transcytosis) of proteins from the basolateral to the apical plasma membrane of hepatocytes. The obtained fraction was enriched in compartments containing known transcytosed proteins and depleted in elements of the secretory pathway, Golgi ...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/0092-8674(91)90210-p
更新日期:1991-01-11 00:00:00
abstract::We have determined the 2.0 A crystal structure of a fragment of human fibronectin encompassing the seventh through the RGD-containing tenth type III repeats (FN7-10). The structure reveals an extended rod-like molecule with a long axis of approximately 140 A and highly variable relationships between adjacent domains. ...
journal_title:Cell
pub_type: 杂志文章
doi:10.1016/s0092-8674(00)81002-8
更新日期:1996-01-12 00:00:00
abstract::Finding a cell that reprograms in a nonstochastic manner without genetic manipulation has proven elusive. In this issue, Guo et al. report the identification of a cell defined by an ultrafast cycle whose progeny reprogram in a synchronous and rapid manner. ...
journal_title:Cell
pub_type: 评论,杂志文章
doi:10.1016/j.cell.2014.01.046
更新日期:2014-02-13 00:00:00