当前位置： SCI文献检索 > PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA期刊下所有文献 > Short telomeres are a risk factor for idiopathic pulmonary fibrosis.

Short telomeres are a risk factor for idiopathic pulmonary fibrosis.

Abstract：

:Idiopathic interstitial pneumonias (IIPs) have a progressive and often fatal course, and their enigmatic etiology has complicated approaches to effective therapies. Idiopathic pulmonary fibrosis (IPF) is the most common of IIPs and shares with IIPs an increased incidence with age and unexplained scarring in the lung. Short telomeres limit tissue renewal capacity in the lung and germ-line mutations in telomerase components, hTERT and hTR, underlie inheritance in a subset of families with IPF. To examine the hypothesis that short telomeres contribute to disease risk in sporadic IIPs, we recruited patients who have no family history and examined telomere length in leukocytes and in alveolar cells. To screen for mutations, we sequenced hTERT and hTR. We also reviewed the cases for features of a telomere syndrome. IIP patients had shorter leukocyte telomeres than age-matched controls (P < 0.0001). In a subset (10%), IIP patients had telomere lengths below the first percentile for their age. Similar to familial cases with mutations, IPF patients had short telomeres in alveolar epithelial cells (P < 0.0001). Although telomerase mutations were rare, detected in 1 of 100 patients, we identified a cluster of individuals (3%) with IPF and cryptogenic liver cirrhosis, another feature of a telomere syndrome. Short telomeres are thus a signature in IIPs and likely play a role in their age-related onset. The clustering of cryptogenic liver cirrhosis with IPF suggests that the telomere shortening we identify has consequences and can contribute to what appears clinically as idiopathic progressive organ failure in the lung and the liver.

journal_name

Proc Natl Acad Sci U S A

journal_title

Proceedings of the National Academy of Sciences of the United States of America

authors

Alder JK,Chen JJ,Lancaster L,Danoff S,Su SC,Cogan JD,Vulto I,Xie M,Qi X,Tuder RM,Phillips JA 3rd,Lansdorp PM,Loyd JE,Armanios MY

doi

10.1073/pnas.0804280105

subject

Has Abstract

pub_date

2008-09-02 00:00:00

pages

13051-6

issue

35

eissn

0027-8424

issn

1091-6490

pii

0804280105

journal_volume

105

pub_type

杂志文章

相关文献

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA文献大全
  • VHL loss in renal cell carcinoma leads to up-regulation of CUB domain-containing protein 1 to stimulate PKC{delta}-driven migration.

    abstract：:A common genetic mutation found in clear cell renal cell carcinoma (CC-RCC) is the loss of the von Hippel-Lindau (VHL) gene, which results in stabilization of hypoxia-inducible factors (HIFs), and contributes to cancer progression and metastasis. CUB-domain-containing protein 1 (CDCP1) was shown to promote metastasis ...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1011777108

    authors： Razorenova OV,Finger EC,Colavitti R,Chernikova SB,Boiko AD,Chan CK,Krieg A,Bedogni B,LaGory E,Weissman IL,Broome-Powell M,Giaccia AJ

    更新日期：2011-02-01 00:00:00

  • Intestinal trefoil factor confers colonic epithelial resistance to apoptosis.

    abstract：:Intestinal trefoil factor (ITF) is an essential regulator of colonic epithelial restitution, the rapid migration of colonocytes over mucosal wounds. High levels of ITF are frequently present in colorectal cancers and derived cell lines. Mucosal restitution requires the detachment of epithelium from substrate, which wo...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.97.2.799

    authors： Taupin DR,Kinoshita K,Podolsky DK

    更新日期：2000-01-18 00:00:00

  • Attosecond electron pulses for 4D diffraction and microscopy.

    abstract：:In this contribution, we consider the advancement of ultrafast electron diffraction and microscopy to cover the attosecond time domain. The concept is centered on the compression of femtosecond electron packets to trains of 15-attosecond pulses by the use of the ponderomotive force in synthesized gratings of optical f...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.0709019104

    authors： Baum P,Zewail AH

    更新日期：2007-11-20 00:00:00

  • The tRNA processing enzyme RNase T is essential for maturation of 5S RNA.

    abstract：:The maturation of 5S RNA in Escherichia coli is poorly understood. Although it is known that large precursors of 5S RNA accumulate in mutant cells lacking the endoribonuclease-RNase E, almost nothing is known about how the mature 5' and 3' termini of these molecules are generated. We have examined 5S RNA maturation in...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.92.15.6883

    authors： Li Z,Deutscher MP

    更新日期：1995-07-18 00:00:00

  • Alterations in cellular metabolism triggered by URA7 or GLN3 inactivation cause imbalanced dNTP pools and increased mutagenesis.

    abstract：:Eukaryotic DNA replication fidelity relies on the concerted action of DNA polymerase nucleotide selectivity, proofreading activity, and DNA mismatch repair (MMR). Nucleotide selectivity and proofreading are affected by the balance and concentration of deoxyribonucleotide (dNTP) pools, which are strictly regulated by r...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1618714114

    authors： Schmidt TT,Reyes G,Gries K,Ceylan CÜ,Sharma S,Meurer M,Knop M,Chabes A,Hombauer H

    更新日期：2017-05-30 00:00:00

  • DNA polymerases δ and λ cooperate in repairing double-strand breaks by microhomology-mediated end-joining in Saccharomyces cerevisiae.

    abstract：:Maintenance of genome stability is carried out by a suite of DNA repair pathways that ensure the repair of damaged DNA and faithful replication of the genome. Of particular importance are the repair pathways, which respond to DNA double-strand breaks (DSBs), and how the efficiency of repair is influenced by sequence h...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1507833112

    authors： Meyer D,Fu BX,Heyer WD

    更新日期：2015-12-15 00:00:00

  • Responses to pup vocalizations in subordinate naked mole-rats are induced by estradiol ingested through coprophagy of queen's feces.

    abstract：:Naked mole-rats form eusocial colonies consisting of a single breeding female (the queen), several breeding males, and sexually immature adults (subordinates). Subordinates are cooperative and provide alloparental care by huddling and retrieving pups to the nest. However, the physiological mechanism(s) underlying allo...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1720530115

    authors： Watarai A,Arai N,Miyawaki S,Okano H,Miura K,Mogi K,Kikusui T

    更新日期：2018-09-11 00:00:00

  • Processing of the 5' end of Escherichia coli 16S ribosomal RNA.

    abstract：:We have isolated and partially characterized an endonuclease involved in processing the 5' end of 16S rRNA of Escherichia coli. A mutant strain that is deficient in this enzyme accumulates a new precursor of 16S rRNA, named 16.3S rRNA. This rRNA has the 3' end of mature 16S rRNA but is about 60 nucleotides longer at t...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.75.8.3598

    authors： Dahlberg AE,Dahlberg JE,Lund E,Tokimatsu H,Rabson AB,Calvert PC,Reynolds F,Zahalak M

    更新日期：1978-08-01 00:00:00

  • Global transcriptional repression: An initial and essential step for Plasmodium sexual development.

    abstract：:Gametocytes are nonreplicative sexual forms that mediate malaria transmission to a mosquito vector. They are generated from asexual blood-stage parasites that proliferate in the circulation. However, little is known about how this transition is genetically regulated. Here, we report that an Apetala2 (AP2) family trans...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1504389112

    authors： Yuda M,Iwanaga S,Kaneko I,Kato T

    更新日期：2015-10-13 00:00:00

  • Evolution of Antp-class genes and differential expression of Hydra Hox/paraHox genes in anterior patterning.

    abstract：:The conservation of developmental functions exerted by Antp-class homeoproteins in protostomes and deuterostomes suggested that homologs with related functions are present in diploblastic animals. Our phylogenetic analyses showed that Antp-class homeodomains belong either to non-Hox or to Hox/paraHox families. Among t...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.97.9.4493

    authors： Gauchat D,Mazet F,Berney C,Schummer M,Kreger S,Pawlowski J,Galliot B

    更新日期：2000-04-25 00:00:00

  • Catheterization alters bladder ecology to potentiate Staphylococcus aureus infection of the urinary tract.

    abstract：:Methicillin-resistant Staphylococcus aureus (MRSA) is an emerging cause of catheter-associated urinary tract infection (CAUTI), which frequently progresses to more serious invasive infections. We adapted a mouse model of CAUTI to investigate how catheterization increases an individual's susceptibility to MRSA UTI. Thi...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1707572114

    authors： Walker JN,Flores-Mireles AL,Pinkner CL,Schreiber HL 4th,Joens MS,Park AM,Potretzke AM,Bauman TM,Pinkner JS,Fitzpatrick JAJ,Desai A,Caparon MG,Hultgren SJ

    更新日期：2017-10-10 00:00:00

  • Microtubule-associated protein 2 kinases, ERK1 and ERK2, undergo autophosphorylation on both tyrosine and threonine residues: implications for their mechanism of activation.

    abstract：:Microtubule-associated protein 2 kinase (MAP kinase), which exists in several forms, is a protein serine/threonine kinase that participates in a growth factor-activated protein kinase cascade in which it activates a ribosomal protein S6 kinase (pp90rsk) while being regulated itself by a cytoplasmic factor (MAP kinase ...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.88.14.6142

    authors： Seger R,Ahn NG,Boulton TG,Yancopoulos GD,Panayotatos N,Radziejewska E,Ericsson L,Bratlien RL,Cobb MH,Krebs EG

    更新日期：1991-07-15 00:00:00

  • Germ-line expression of an oncogenic erbB2 allele confers resistance to erbB2-induced mammary tumorigenesis.

    abstract：:We have previously shown that mammary epithelial specific expression of the activated erbB2 allele under the control of the endogenous promoter in mice resulted in the formation of mammary adenocarcinomas. To assess whether mammary tumorigenesis in this model is influenced by the developmental window of expression, we...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.0306802101

    authors： Andrechek ER,Hardy WR,Laing MA,Muller WJ

    更新日期：2004-04-06 00:00:00

  • Persistence of dormant tumor cells in the bone marrow of tumor cell-vaccinated mice correlates with long-term immunological protection.

    abstract：:Live proliferation-competent and irradiated proliferation-incompetent L5178 murine lymphoma cells (Eb cell line) were compared for their potency to induce systemic anti-tumor immunity in syngeneic DBA/2 mice. The tumorigenic potential in vivo of live Eb cells was suppressed through local secretion of interleukin 4 (IL...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.91.16.7430

    authors： Khazaie K,Prifti S,Beckhove P,Griesbach A,Russell S,Collins M,Schirrmacher V

    更新日期：1994-08-02 00:00:00

  • Metabotropic glutamate receptor 3 activation is required for long-term depression in medial prefrontal cortex and fear extinction.

    abstract：:Clinical studies have revealed that genetic variations in metabotropic glutamate receptor 3 (mGlu3) affect performance on cognitive tasks dependent upon the prefrontal cortex (PFC) and may be linked to psychiatric conditions such as schizophrenia, bipolar disorder, and addiction. We have performed a series of studies ...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1416196112

    authors： Walker AG,Wenthur CJ,Xiang Z,Rook JM,Emmitte KA,Niswender CM,Lindsley CW,Conn PJ

    更新日期：2015-01-27 00:00:00

  • Synthesis and assembly of self-complementary calix[4]arenes.

    abstract：:A calix[4]arene was designed to reversibly dimerize and form an egg-shaped enclosure. Adhesive interactions in the assembly were provided by four self-associating ureas, which form a cyclic array containing 16 hydrogen bonds. The synthesis was completed in four steps from the previously described O,O',O",O"'-tetrabenz...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.92.26.12403

    authors： Shimizu KD,Rebek J Jr

    更新日期：1995-12-19 00:00:00

  • Isolation and characterization of human cDNA clones encoding a high mobility group box protein that recognizes structural distortions to DNA caused by binding of the anticancer agent cisplatin.

    abstract：:Human cDNA clones encoding a structure-specific recognition protein, SSRP1, that binds specifically to DNA modified with cisplatin have been isolated and characterized. The SSRP1 gene maps to human chromosome 11q12. The cDNA clones, obtained by using partial-length cDNAs described previously, predict an 81-kDa protein...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.89.6.2307

    authors： Bruhn SL,Pil PM,Essigmann JM,Housman DE,Lippard SJ

    更新日期：1992-03-15 00:00:00

  • The evolution of N-glycan-dependent endoplasmic reticulum quality control factors for glycoprotein folding and degradation.

    abstract：:Asn-linked glycans (N-glycans) play important roles in the quality control (QC) of glycoprotein folding in the endoplasmic reticulum (ER) lumen and in ER-associated degradation (ERAD) of proteins by cytosolic proteasomes. A UDP-Glc:glycoprotein glucosyltransferase glucosylates N-glycans of misfolded proteins, which ar...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.0704862104

    authors： Banerjee S,Vishwanath P,Cui J,Kelleher DJ,Gilmore R,Robbins PW,Samuelson J

    更新日期：2007-07-10 00:00:00

  • On the promotion of human flourishing.

    abstract：:Many empirical studies throughout the social and biomedical sciences focus only on very narrow outcomes such as income, or a single specific disease state, or a measure of positive affect. Human well-being or flourishing, however, consists in a much broader range of states and outcomes, certainly including mental and ...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1702996114

    authors： VanderWeele TJ

    更新日期：2017-08-01 00:00:00

  • Aberrant miR-182 expression promotes melanoma metastasis by repressing FOXO3 and microphthalmia-associated transcription factor.

    abstract：:The highly aggressive character of melanoma makes it an excellent model for probing the mechanisms underlying metastasis, which remains one of the most difficult challenges in treating cancer. We find that miR-182, member of a miRNA cluster in a chromosomal locus (7q31-34) frequently amplified in melanoma, is commonly...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.0808263106

    authors： Segura MF,Hanniford D,Menendez S,Reavie L,Zou X,Alvarez-Diaz S,Zakrzewski J,Blochin E,Rose A,Bogunovic D,Polsky D,Wei J,Lee P,Belitskaya-Levy I,Bhardwaj N,Osman I,Hernando E

    更新日期：2009-02-10 00:00:00

  • Why we need to look beyond the glass transition temperature to characterize the dynamics of thin supported polymer films.

    abstract：:There is significant variation in the reported magnitude and even the sign of [Formula: see text] shifts in thin polymer films with nominally the same chemistry, film thickness, and supporting substrate. The implicit assumption is that methods used to estimate [Formula: see text] in bulk materials are relevant for inf...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1722024115

    authors： Zhang W,Douglas JF,Starr FW

    更新日期：2018-05-29 00:00:00

  • Monitoring of cytosolic free Ca2+ in C5a-stimulated neutrophils: loss of receptor-modulated Ca2+ stores and Ca2+ uptake in granule-free cytoplasts.

    abstract：:The cytosolic concentration of free Ca2+ in bovine neutrophils was monitored by using the intracellular Ca2+ indicator quin2, 2-[[2-bis(acetylamino)-5-methylphenoxy]methyl-6-methoxy-8- bis(acetylamino)]quinoline. Neutrophils at rest have a cytosolic Ca2+ concentration of 85 +/- 5 nM, which in 2-4 min increases to 300-...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.81.5.1416

    authors： Gennaro R,Pozzan T,Romeo D

    更新日期：1984-03-01 00:00:00

  • Anticonvulsant activity of a nonpeptide galanin receptor agonist.

    abstract：:Galanin is a neuropeptide with a wide variety of biological functions, including that of a strong endogenous anticonvulsant. No nonpeptide ligands, capable of activating galanin receptors, are available today. Based on known pharmacophores of galanin, a combinatorial library was designed, synthesized, and screened at ...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.102163499

    authors： Saar K,Mazarati AM,Mahlapuu R,Hallnemo G,Soomets U,Kilk K,Hellberg S,Pooga M,Tolf BR,Shi TS,Hökfelt T,Wasterlain C,Bartfai T,Langel U

    更新日期：2002-05-14 00:00:00

  • Habituation of tobacco pith cells for factors promoting cell division is heritable and potentially reversible.

    abstract：:A type of heritable cellular change, known as habituation, occurs spontaneously in plant tissue and cell culture. This phenomenon is characterized by a newly acquired capacity of plant cells to produce growth regulatory substances. Using cloned lines of tobacco pith parenchyma cells, we demonstrated that a newly acqui...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.70.9.2660

    authors： Binns A,Meins F

    更新日期：1973-09-01 00:00:00

  • Targeted mutagenesis of Lis1 disrupts cortical development and LIS1 homodimerization.

    abstract：:Lissencephaly is a severe brain malformation in humans. To study the function of the gene mutated in lissencephaly (LIS1), we deleted the first coding exon from the mouse Lis1 gene. The deletion resulted in a shorter protein (sLIS1) that initiates from the second methionine, a unique situation because most LIS1 mutati...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.101122598

    authors： Cahana A,Escamez T,Nowakowski RS,Hayes NL,Giacobini M,von Holst A,Shmueli O,Sapir T,McConnell SK,Wurst W,Martinez S,Reiner O

    更新日期：2001-05-22 00:00:00

  • B850 pigment-protein complex of Rhodopseudomonas sphaeroides: Extinction coefficients, circular dichroism, and the reversible binding of bacteriochlorophyll.

    abstract：:Chromatophores of Rhodopseudomonas sphaeroides yield the antenna complex B850 in either of two states, depending on the method of isolation. Methods using dodecyl (= lauryl) dimethylamine oxide yield B850 with an absorption spectrum like that in vivo: the bands at 800 and 850 nm, due to the bacteriochlorophyll (Bchl) ...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.78.9.5583

    authors： Clayton RK,Clayton BJ

    更新日期：1981-09-01 00:00:00

  • Psychopathic individuals exhibit but do not avoid regret during counterfactual decision making.

    abstract：:Psychopathy is associated with persistent antisocial behavior and a striking lack of regret for the consequences of that behavior. Although explanatory models for psychopathy have largely focused on deficits in affective responsiveness, recent work indicates that aberrant value-based decision making may also play a ro...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1609985113

    authors： Baskin-Sommers A,Stuppy-Sullivan AM,Buckholtz JW

    更新日期：2016-12-13 00:00:00

  • Personality predicts activity in reward and emotional regions associated with humor.

    abstract：:Previous research and theory suggest that two stable personality dimensions, extroversion and neuroticism, differentially influence emotional reactivity to a variety of pleasurable phenomena. Here, we use event-related functional MRI to address the putative neural and behavioral associations between humor appreciation...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.0408457102

    authors： Mobbs D,Hagan CC,Azim E,Menon V,Reiss AL

    更新日期：2005-11-08 00:00:00

  • Spironolactone blocks Epstein-Barr virus production by inhibiting EBV SM protein function.

    abstract：:Clinically available drugs active against Epstein-Barr virus (EBV) and other human herpesviruses are limited to those targeting viral DNA replication. To identify compounds directed against other steps in the viral life cycle, we searched for drugs active against the EBV SM protein, which is essential for infectious v...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.1523686113

    authors： Verma D,Thompson J,Swaminathan S

    更新日期：2016-03-29 00:00:00

  • p70S6 kinase signals cell survival as well as growth, inactivating the pro-apoptotic molecule BAD.

    abstract：:Cytokines often deliver simultaneous, yet distinct, cell growth and cell survival signals. The 70-kDa ribosomal protein S6 kinase (p70S6K) is known to regulate cell growth by inducing protein synthesis components. We purified membrane-based p70S6K as a kinase responsible for site-specific phosphorylation of BAD, which...

    journal_title：Proceedings of the National Academy of Sciences of the United States of America

    pub_type： 杂志文章

    doi：10.1073/pnas.171301998

    authors： Harada H,Andersen JS,Mann M,Terada N,Korsmeyer SJ

    更新日期：2001-08-14 00:00:00