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 > Dystrophin protects the sarcolemma from stresses developed during muscle contraction.

Dystrophin protects the sarcolemma from stresses developed during muscle contraction.

Abstract：

:The protein dystrophin, normally found on the cytoplasmic surface of skeletal muscle cell membranes, is absent in patients with Duchenne muscular dystrophy as well as mdx (X-linked muscular dystrophy) mice. Although its primary structure has been determined, the precise functional role of dystrophin remains the subject of speculation. In the present study, we demonstrate that dystrophin-deficient muscle fibers of the mdx mouse exhibit an increased susceptibility to contraction-induced sarcolemmal rupture. The level of sarcolemmal damage is directly correlated with the magnitude of mechanical stress placed upon the membrane during contraction rather than the number of activations of the muscle. These findings strongly support the proposition that the primary function of dystrophin is to provide mechanical reinforcement to the sarcolemma and thereby protect it from the membrane stresses developed during muscle contraction. Furthermore, the methodology used in this study should prove useful in assessing the efficacy of dystrophin gene therapy in the mdx mouse.

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

Petrof BJ,Shrager JB,Stedman HH,Kelly AM,Sweeney HL

doi

10.1073/pnas.90.8.3710

subject

Has Abstract

pub_date

1993-04-15 00:00:00

pages

3710-4

issue

8

eissn

0027-8424

issn

1091-6490

journal_volume

90

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
  • Isolation and characterization of DNA sequences amplified in multidrug-resistant hamster cells.

    abstract：:The mechanism by which mammalian cells acquire resistance to chemotherapeutic agents has been investigated by using molecular genetic techniques. LZ and C5, two independently derived multidrug-resistant Chinese hamster cell lines, share specific amplified DNA sequences. We demonstrate that commonly amplified DNA seque...

    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.83.2.337

    authors： Gros P,Croop J,Roninson I,Varshavsky A,Housman DE

    更新日期：1986-01-01 00:00:00

  • A cell type-selective apoptosis-inducing small molecule for the treatment of brain cancer.

    abstract：:Glioblastoma multiforme (GBM; grade IV astrocytoma) is the most prevalent and aggressive form of primary brain cancer. A subpopulation of multipotent cells termed GBM cancer stem cells (CSCs) play a critical role in tumor initiation, tumor maintenance, metastasis, drug resistance, and recurrence following surgery. Her...

    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.1816626116

    authors： Lucki NC,Villa GR,Vergani N,Bollong MJ,Beyer BA,Lee JW,Anglin JL,Spangenberg SH,Chin EN,Sharma A,Johnson K,Sander PN,Gordon P,Skirboll SL,Wurdak H,Schultz PG,Mischel PS,Lairson LL

    更新日期：2019-03-26 00:00:00

  • Attachment of a 40-base-pair G + C-rich sequence (GC-clamp) to genomic DNA fragments by the polymerase chain reaction results in improved detection of single-base changes.

    abstract：:Denaturing gradient gel electrophoresis (DGGE) can be used to distinguish two DNA molecules that differ by as little as a single-base substitution. This method detects approximately 50% of all possible single-base changes in DNA fragments ranging from 50 to approximately 1000 base pairs. To increase the number of sing...

    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.86.1.232

    authors： Sheffield VC,Cox DR,Lerman LS,Myers RM

    更新日期：1989-01-01 00:00:00

  • Alpha-actinin2 cytoskeletal protein is required for the functional membrane localization of a Ca2+-activated K+ channel (SK2 channel).

    abstract：:The importance of proper ion channel trafficking is underpinned by a number of channel-linked genetic diseases whose defect is associated with failure to reach the cell surface. Conceptually, it is reasonable to suggest that the function of ion channels depends critically on the precise subcellular localization and th...

    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.0908207106

    authors： Lu L,Timofeyev V,Li N,Rafizadeh S,Singapuri A,Harris TR,Chiamvimonvat N

    更新日期：2009-10-27 00:00:00

  • Cultured melanocytes from dilute mutant mice exhibit dendritic morphology and altered melanosome distribution.

    abstract：:Mutant alleles at the dilute unconventional myosin heavy chain locus cause diluted coat color, opisthotonic seizures, and death. The dilute coat color phenotype is caused by irregular clumping of pigment in the hair, but amounts of melanin are unchanged from wild-type controls. The melanocyte phenotype has been descri...

    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.93.25.14554

    authors： Provance DW Jr,Wei M,Ipe V,Mercer JA

    更新日期：1996-12-10 00:00:00

  • Plasminogen activator secretion by granule neurons in cultures of developing cerebellum.

    abstract：:Dispersed cell cultures were established from 7- to 9-day postnatal mouse cerebellum. The fibrin slice method was used to obtain a localization of plasminogen activator production to specific cells. Fibrinolytically active cells were small (5- to 8-micrometer diameter), round, and occurred singly or in aggregates. Fib...

    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.78.12.7810

    authors： Krystosek A,Seeds NW

    更新日期：1981-12-01 00:00:00

  • Architecture of a minimal signaling pathway explains the T-cell response to a 1 million-fold variation in antigen affinity and dose.

    abstract：:T cells must respond differently to antigens of varying affinity presented at different doses. Previous attempts to map peptide MHC (pMHC) affinity onto T-cell responses have produced inconsistent patterns of responses, preventing formulations of canonical models of T-cell signaling. Here, a systematic analysis of T-c...

    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.1608820113

    authors： Lever M,Lim HS,Kruger P,Nguyen J,Trendel N,Abu-Shah E,Maini PK,van der Merwe PA,Dushek O

    更新日期：2016-10-25 00:00:00

  • Mammalian monogamy is not controlled by a single gene.

    abstract：:Complex social behavior in Microtus voles and other mammals has been postulated to be under the direct genetic control of a single locus: the arginine vasopressin 1a receptor (avpr1a) gene. Using a phylogenetic approach, we show that a repetitive element in the promoter region of avpr1a, which reportedly causes social...

    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.0602380103

    authors： Fink S,Excoffier L,Heckel G

    更新日期：2006-07-18 00:00:00

  • Retinoid X receptor alpha forms tetramers in solution.

    abstract：:Protein-protein interactions allow the retinoid X receptor (RXR) to bind to cognate DNA as a homo- or a heterodimer and to participate in mediating the effects of a variety of hormones on gene transcription. Here we report a systematic study of the oligomeric state of RXR in the absence of a DNA template. We have used...

    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.92.19.8645

    authors： Kersten S,Kelleher D,Chambon P,Gronemeyer H,Noy N

    更新日期：1995-09-12 00:00:00

  • Large-scale evidence of dependency length minimization in 37 languages.

    abstract：:Explaining the variation between human languages and the constraints on that variation is a core goal of linguistics. In the last 20 y, it has been claimed that many striking universals of cross-linguistic variation follow from a hypothetical principle that dependency length--the distance between syntactically related...

    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.1502134112

    authors： Futrell R,Mahowald K,Gibson E

    更新日期：2015-08-18 00:00:00

  • Genotypic analysis of N-ethyl-N-nitrosourea-induced mutations by Taq I restriction fragment length polymorphism/polymerase chain reaction in the c-H-ras1 gene.

    abstract：:In genotypic mutation analysis DNA sequence changes are determined without the in vivo or in vitro selection of phenotypically altered cells. We have studied the induction of base-pair changes by N-ethyl-N-nitrosourea in Taq I endonuclease recognition site 2508-2511 (TCGA) of the c-H-ras1 gene in human fibroblasts by ...

    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.89.12.5331

    authors： Chiocca SM,Sandy MS,Cerutti PA

    更新日期：1992-06-15 00:00:00

  • A phylogenetic analysis of myosin heavy chain type II sequences corroborates that Acoela and Nemertodermatida are basal bilaterians.

    abstract：:Bilateria are currently subdivided into three superclades: Deuterostomia, Ecdysozoa, and Lophotrochozoa. Within this new taxonomic frame, acoelomate Platyhelminthes, for a long time held to be basal bilaterians, are now considered spiralian lophotrochozoans. However, recent 18S rDNA [small subunit (SSU)] analyses have...

    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.172390199

    authors： Ruiz-Trillo I,Paps J,Loukota M,Ribera C,Jondelius U,Baguna J,Riutort M

    更新日期：2002-08-20 00:00:00

  • Energies and kinetics of radical pairs involving bacteriochlorophyll and bacteriopheophytin in bacterial reaction centers.

    abstract：:Absorbance changes reflecting the formation of a transient radical-pair state, P(F), were measured in reaction centers from Rhodopseudomonas sphaeroides under conditions that blocked electron transfer to a later carrier (a quinone, Q). The temperature dependence of the absorbance changes suggests that P(F) is an equil...

    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.78.2.957

    authors： Shuvalov VA,Parson WW

    更新日期：1981-02-01 00:00:00

  • Increased ribosomal accuracy increases a programmed translational frameshift in Escherichia coli.

    abstract：:We have tested the effect of increased ribosomal fidelity on a modified version of the programmed release factor 2 (RF2) translational frameshift. In the constructs tested, the original UGA codon at the site of the shift was replaced by either of two sense codons, UGG (tryptophan), which allows a frameshift of approxi...

    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.90.6.2315

    authors： Sipley J,Goldman E

    更新日期：1993-03-15 00:00:00

  • The catalytic subunit of yeast telomerase.

    abstract：:Telomerase is an RNA-directed DNA polymerase, composed of RNA and protein subunits, that replicates the telomere ends of linear eukaryotic chromosomes. Using a genetic strategy described here, we identify the product of the EST2 gene, Est2p, as a subunit of telomerase in the yeast Saccharomyces cerevisiae. Est2p is re...

    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.94.17.9202

    authors： Counter CM,Meyerson M,Eaton EN,Weinberg RA

    更新日期：1997-08-19 00:00:00

  • Neural correlates of trust.

    abstract：:Trust is a critical social process that helps us to cooperate with others and is present to some degree in all human interaction. However, the underlying brain mechanisms of conditional and unconditional trust in social reciprocal exchange are still obscure. Here, we used hyperfunctional magnetic resonance imaging, 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.0710103104

    authors： Krueger F,McCabe K,Moll J,Kriegeskorte N,Zahn R,Strenziok M,Heinecke A,Grafman J

    更新日期：2007-12-11 00:00:00

  • Mutational analysis of protein phosphatase 2C involved in abscisic acid signal transduction in higher plants.

    abstract：:Protein phosphatase 2C (PP2C) is a class of ubiquitous and evolutionarily conserved serine/threonine PP involved in stress responses in yeasts, mammals, and plants. Here, I present mutational analysis of two Arabidopsis thaliana PP2Cs, encoded by ABI1 and AtPP2C, involved in the plant stress hormone abscisic acid (ABA...

    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.95.3.975

    authors： Sheen J

    更新日期：1998-02-03 00:00:00

  • Accurately quantifying low-abundant targets amid similar sequences by revealing hidden correlations in oligonucleotide microarray data.

    abstract：:Microarrays have enabled the determination of how thousands of genes are expressed to coordinate function within single organisms. Yet applications to natural or engineered communities where different organisms interact to produce complex properties are hampered by theoretical and technological limitations. Here we de...

    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.0601476103

    authors： Marcelino LA,Backman V,Donaldson A,Steadman C,Thompson JR,Preheim SP,Lien C,Lim E,Veneziano D,Polz MF

    更新日期：2006-09-12 00:00:00

  • Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110.

    abstract：:Recent studies have indicated that mammalian cells contain a cytosolic protein disaggregation machinery comprised of Hsc70, DnaJ homologs, and Hsp110 proteins, the last of which acts to accelerate a rate-limiting step of nucleotide exchange of Hsc70. We tested the ability of transgenic overexpression of a Thy1 promote...

    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.1604885113

    authors： Nagy M,Fenton WA,Li D,Furtak K,Horwich AL

    更新日期：2016-05-10 00:00:00

  • Inhibition of prostate cancer cell growth by second-site androgen receptor antagonists.

    abstract：:The impact of ligand binding on nuclear receptor (NR) structure and the ability of target cells to distinguish between different receptor-ligand complexes are key determinants of the pharmacological activity of NR ligands. However, until relatively recently, these mechanistic insights have not been used in a prospecti...

    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.0900185106

    authors： Joseph JD,Wittmann BM,Dwyer MA,Cui H,Dye DA,McDonnell DP,Norris JD

    更新日期：2009-07-21 00:00:00

  • Regulation of zinc homeostasis by inducible NO synthase-derived NO: nuclear metallothionein translocation and intranuclear Zn2+ release.

    abstract：:Zn2+ is critical for the functional and structural integrity of cells and contributes to a number of important processes including gene expression. It has been shown that NO exogenously applied via NO donors resulting in nitrosative stress leads to cytoplasmic Zn2+ release from the zinc storing protein metallothionein...

    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.2335190100

    authors： Spahl DU,Berendji-Grün D,Suschek CV,Kolb-Bachofen V,Kröncke KD

    更新日期：2003-11-25 00:00:00

  • A single amino acid difference in the host APOBEC3G protein controls the primate species specificity of HIV type 1 virion infectivity factor.

    abstract：:The HIV type 1 (HIV-1) virion infectivity factor (Vif) protein blocks the action of the host defense factor APOBEC3G in human cells, thereby allowing release of infectious virions, but fails to inhibit similar APOBEC3G proteins present in some simian cells. Conversely, the Vif protein encoded by the African green monk...

    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.0307713101

    authors： Bogerd HP,Doehle BP,Wiegand HL,Cullen BR

    更新日期：2004-03-16 00:00:00

  • Deactivation kinetics of acid-sensing ion channel 1a are strongly pH-sensitive.

    abstract：:Acid-sensing ion channels (ASICs) are trimeric cation-selective ion channels activated by protons in the physiological range. Recent reports have revealed that postsynaptically localized ASICs contribute to the excitatory postsynaptic current by responding to the transient acidification of the synaptic cleft that acco...

    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.1620508114

    authors： MacLean DM,Jayaraman V

    更新日期：2017-03-21 00:00:00

  • Genes in one megabase of the HLA class I region.

    abstract：:To define the gene content of the HLA class I region, cDNA selection was applied to three overlapping yeast artificial chromosomes (YACs) that spanned 1 megabase (Mb) of this region of the human major histocompatibility complex. These YACs extended from the region centromeric to HLA-E to the region telomeric to HLA-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.90.24.11870

    authors： Wei H,Fan WF,Xu H,Parimoo S,Shukla H,Chaplin DD,Weissman SM

    更新日期：1993-12-15 00:00:00

  • Use of monoclonal antibodies in the purification and characterization of 1-aminocyclopropane-1-carboxylate synthase, an enzyme in ethylene biosynthesis.

    abstract：:1-Aminocyclopropane-1-carboxylate (ACC) synthase (EC 4.4.1.14), extracted from tomato pericarp tissue, was purified 6500-fold by conventional and high-performance liquid chromatography. Two-dimensional gel electrophoresis of this preparation indicated that ACC synthase activity was associated with a protein band at 50...

    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.83.20.7755

    authors： Bleecker AB,Kenyon WH,Somerville SC,Kende H

    更新日期：1986-10-01 00:00:00

  • Synonymous codon substitutions perturb cotranslational protein folding in vivo and impair cell fitness.

    abstract：:In the cell, proteins are synthesized from N to C terminus and begin to fold during translation. Cotranslational folding mechanisms are therefore linked to elongation rate, which varies as a function of synonymous codon usage. However, synonymous codon substitutions can affect many distinct cellular processes, which 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.1907126117

    authors： Walsh IM,Bowman MA,Soto Santarriaga IF,Rodriguez A,Clark PL

    更新日期：2020-02-18 00:00:00

  • Reforestation as a novel abatement and compliance measure for ground-level ozone.

    abstract：:High ambient ozone (O3) concentrations are a widespread and persistent problem globally. Although studies have documented the role of forests in removing O3 and one of its precursors, nitrogen dioxide (NO2), the cost effectiveness of using peri-urban reforestation for O3 abatement purposes has not been examined. We de...

    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.1409785111

    authors： Kroeger T,Escobedo FJ,Hernandez JL,Varela S,Delphin S,Fisher JR,Waldron J

    更新日期：2014-10-07 00:00:00

  • Diverse karyotypic abnormalities of the c-myc locus associated with c-myc dysregulation and tumor progression in multiple myeloma.

    abstract：:Translocations involving c-myc and an Ig locus have been reported rarely in human multiple myeloma (MM). Using specific fluorescence in situ hybridization probes, we show complex karyotypic abnormalities of the c-myc or L-myc locus in 19 of 20 MM cell lines and approximately 50% of advanced primary MM tumors. These ab...

    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.97.1.228

    authors： Shou Y,Martelli ML,Gabrea A,Qi Y,Brents LA,Roschke A,Dewald G,Kirsch IR,Bergsagel PL,Kuehl WM

    更新日期：2000-01-04 00:00:00

  • Familial hemiplegic migraine mutations increase Ca(2+) influx through single human CaV2.1 channels and decrease maximal CaV2.1 current density in neurons.

    abstract：:Insights into the pathogenesis of migraine with aura may be gained from a study of human Ca(V)2.1 channels containing mutations linked to familial hemiplegic migraine (FHM). Here, we extend the previous single-channel analysis to human Ca(V)2.1 channels containing mutation V1457L. This mutation increased the channel o...

    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.192242399

    authors： Tottene A,Fellin T,Pagnutti S,Luvisetto S,Striessnig J,Fletcher C,Pietrobon D

    更新日期：2002-10-01 00:00:00

  • Subcellular localization of mineralocorticoid receptors in living cells: effects of receptor agonists and antagonists.

    abstract：:Results on the subcellular localization of the mineralocorticoid receptor (MR) have been controversial. To determine the subcellular distribution and trafficking of the MR in living cells after binding of agonists and antagonists, we expressed a MR-green fluorescent protein (GFP) chimera in mammalian cells lacking end...

    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.95.6.2973

    authors： Fejes-Tóth G,Pearce D,Náray-Fejes-Tóth A

    更新日期：1998-03-17 00:00:00