Generation and rescue of a murine model of platelet dysfunction: the Bernard-Soulier syndrome.

abstract：:It is a well known fact that stirring keeps particles suspended in fluids. This is apparent, for instance, when shaking medicine flasks, when agitating tea deposits in a mug, or when heavy winds fill the air with dust particles. The commonplace nature of such observations makes it easy to accept that this feature will...

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

authors： Ruiz J,Macías D,Peters F

更新日期：2004-12-21 00:00:00

abstract：:Light activates rod outer segment (ROS) phosphodiesterase (PDEase), as shown by previous biochemical and physiological studies. We have further investigated the role of PDEase in this system by injecting trypsin-activated PDEase, purified from bovine ROS, into single ROS of the isolated retina of the toad Bufo marinus...

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

authors： Shimoda Y,Hurley JB,Miller WH

更新日期：1984-01-01 00:00:00

abstract：:Nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disorder and is strongly associated with obesity and type 2 diabetes. Currently, there is no approved pharmacological treatment for this disease, but improvement of insulin resistance using peroxisome proliferator-activated receptor-γ (PPARγ) ag...

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

authors： Liu W,Struik D,Nies VJ,Jurdzinski A,Harkema L,de Bruin A,Verkade HJ,Downes M,Evans RM,van Zutphen T,Jonker JW

更新日期：2016-02-23 00:00:00

abstract：:X-chromosome inactivation (XCI) is initiated by the long noncoding RNA Xist, which coats the inactive X (Xi) and targets Polycomb repressive complex 2 (PRC2) in cis. Epigenomic analyses have provided significant insight into Xist binding patterns and chromatin organization of the Xi. However, such epigenomic analyses ...

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

authors： Sunwoo H,Wu JY,Lee JT

更新日期：2015-08-04 00:00:00

abstract：:The T-cell receptor (TCR) gamma gene product occurs in association with T3 (CD3) polypeptides on the surface of human T lymphocytes. TCR gamma lymphocytes express arrays of T3 polypeptides distinct from those typically observed on TCR alpha beta lymphocytes. This report demonstrates that identical T3 gamma, delta, and...

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

authors： Krangel MS,Bierer BE,Devlin P,Clabby M,Strominger JL,McLean J,Brenner MB

更新日期：1987-06-01 00:00:00

abstract：:Methylmalonyl-CoA mutase (2-methylmalonyl-CoA CoA-carbonylmutase, EC 5.4.99.2) is a mitochondrial enzyme whose deficiency in man leads to several biochemically and clinically heterogenous++ forms of methylmalonic acidemia. Intact fibroblasts from 21 patients with mutase apoenzyme deficiency have been pulse-labeled wit...

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

authors： Fenton WA,Hack AM,Kraus JP,Rosenberg LE

更新日期：1987-03-01 00:00:00

abstract：:Direct cell-cell communication is crucial for many processes in biology, particularly embryogenesis, interactions between hematopoetic cells, and in the nervous system. This communication is often mediated by the binding of receptors to cognate ligands at a cell-cell junction. One such interaction that is very importa...

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

authors： Qi S,Krogsgaard M,Davis MM,Chakraborty AK

更新日期：2006-03-21 00:00:00

abstract：:Although potassium channels play a variety of roles in shaping the electrical properties of neurons, little is known about how these channels are constituted in neurons. To examine the assembly and physiological function of A-type K+ channels in mature differentiated neurons, we have developed a highly efficient gene ...

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

authors： Kaang BK,Pfaffinger PJ,Grant SG,Kandel ER,Furukawa Y

更新日期：1992-02-01 00:00:00

abstract：:We describe here a third region of variability in human fibronectin (FN) due to alternative RNA splicing. Two other positions of alternative splicing have been reported previously (ED and IIICS). The third region involves a 273-nucleotide exon encoding exactly one 91-amino acid repeat of type III homology, located bet...

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

authors： Gutman A,Kornblihtt AR

更新日期：1987-10-01 00:00:00

abstract：:To explore protein space from a global perspective, we consider 9,710 SCOP (Structural Classification of Proteins) domains with up to 70% sequence identity and present all similarities among them as networks: In the "domain network," nodes represent domains, and edges connect domains that share "motifs," i.e., signifi...

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

authors： Nepomnyachiy S,Ben-Tal N,Kolodny R

更新日期：2014-08-12 00:00:00

abstract：:The light-harvesting chlorophyll a/b protein (LHCP) is an integral thylakoid membrane protein. It is made in the cytosol as a precursor (pLHCP), imported into chloroplasts, and subsequently integrated into thylakoids. Integration of pLHCP into thylakoids requires a stromal protein factor that functions in part to main...

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

authors： Yuan J,Henry R,Cline K

更新日期：1993-09-15 00:00:00

abstract：:HD domain phosphohydrolase enzymes are characterized by a conserved set of histidine and aspartate residues that coordinate an active site metallocenter. Despite the important roles these enzymes play in nucleotide metabolism and signal transduction, few have been both biochemically and structurally characterized. Her...

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

authors： Bridwell-Rabb J,Kang G,Zhong A,Liu HW,Drennan CL

更新日期：2016-11-29 00:00:00

abstract：:Bufonid anurans of the genus Atelopus contain both steroidal bufadienolides and various guanidinium alkaloids of the tetrodotoxin class. The former inhibit sodium-potassium ATPases, whereas the latter block voltage-dependent sodium channels. The structure of one guanidinium alkaloid, zetekitoxin AB, has remained a mys...

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

authors： Yotsu-Yamashita M,Kim YH,Dudley SC Jr,Choudhary G,Pfahnl A,Oshima Y,Daly JW

更新日期：2004-03-30 00:00:00

abstract：:We tested the influence of a photothrombotic lesion in somatosensory cortex on plasticity in the mouse visual system and the efficacy of anti-inflammatory treatment to rescue compromised learning. To challenge plasticity mechanisms, we induced monocular deprivation (MD) in 3-mo-old mice. In control animals, MD induced...

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

authors： Greifzu F,Schmidt S,Schmidt KF,Kreikemeier K,Witte OW,Löwel S

更新日期：2011-09-13 00:00:00

abstract：:The Wnts are a family of glycoproteins that regulate cell proliferation, fate decisions, and differentiation. In our study, we examined the contribution of Wnts to the development of ventral midbrain (VM) dopaminergic (DA) neurons. Our results show that beta-catenin is expressed in DA precursor cells and that beta-cat...

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

authors： Castelo-Branco G,Wagner J,Rodriguez FJ,Kele J,Sousa K,Rawal N,Pasolli HA,Fuchs E,Kitajewski J,Arenas E

更新日期：2003-10-28 00:00:00

abstract：:We have selected from a mixture of random polynucleotides an RNA that is able to replicate in the presence of the DNA-dependent RNA polymerase of Escherichia coli, provided the medium contains Mn(2+) and ITP, in addition to ATP, CTP, and UTP. The RNA consists of a chain of about 125 (+/-25) nucleotides and appears to ...

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

authors： Biebricher CK,Orgel LE

更新日期：1973-03-01 00:00:00

abstract：:For many intraoperative decisions surgeons depend on frozen section pathology, a technique developed over 150 y ago. Technical innovations that permit rapid molecular characterization of tissue samples at the time of surgery are needed. Here, using desorption electrospray ionization (DESI) MS, we rapidly detect the tu...

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

authors： Santagata S,Eberlin LS,Norton I,Calligaris D,Feldman DR,Ide JL,Liu X,Wiley JS,Vestal ML,Ramkissoon SH,Orringer DA,Gill KK,Dunn IF,Dias-Santagata D,Ligon KL,Jolesz FA,Golby AJ,Cooks RG,Agar NY

更新日期：2014-07-29 00:00:00

abstract：:The hydrodynamic properties of the detergent-solubilized, terminal membrane complex of serum complement components C5-C9 [C5b-9(m)] were studied to obtain an estimate of its molecular weight. In a solution of Triton X-100/deoxycholate, the protein complex binds 17% Triton X-100 and 11% deoxycholate by weight. The sedi...

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

authors： Bhakdi S,Tranum-Jensen J

更新日期：1981-03-01 00:00:00

abstract：:In many song birds, males develop their songs as adults by imitating the songs of one or more tutors, memorized previously during a sensitive phase early in life. Previous work using two assays, the production of imitations by adult males and playback-induced calling by young birds during the sensitive phase for memor...

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

authors： Nelson DA

更新日期：2000-11-21 00:00:00

abstract：:P2X receptors for ATP have a wide range of physiological roles and comprise a structurally distinct family of ligand-gated trimeric ion channels. The crystal structure of a P2X4 receptor, in combination with mutagenesis studies, has provided a model of the intersubunit ATP-binding sites and identified an extracellular...

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

authors： Roberts JA,Allsopp RC,El Ajouz S,Vial C,Schmid R,Young MT,Evans RJ

更新日期：2012-03-20 00:00:00

abstract：:Lymphangioleiomyomatosis (LAM) is an often fatal disease primarily affecting young women in which tuberin (TSC2)-null cells metastasize to the lungs. The mechanisms underlying the striking female predominance of LAM are unknown. We report here that 17-beta-estradiol (E(2)) causes a 3- to 5-fold increase in pulmonary m...

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

authors： Yu JJ,Robb VA,Morrison TA,Ariazi EA,Karbowniczek M,Astrinidis A,Wang C,Hernandez-Cuebas L,Seeholzer LF,Nicolas E,Hensley H,Jordan VC,Walker CL,Henske EP

更新日期：2009-02-24 00:00:00

abstract：:Point mutations in either of the two nucleotide-binding domains (NBD) of Hsp104 (NBD1 and NBD2) eliminate its thermotolerance function in vivo. In vitro, NBD1 mutations virtually eliminate ATP hydrolysis with little effect on hexamerization; analogous NBD2 mutations reduce ATPase activity and severely impair hexameriz...

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

authors： Schirmer EC,Ware DM,Queitsch C,Kowal AS,Lindquist SL

更新日期：2001-01-30 00:00:00

abstract：:Male chickens with the henny feathering trait have a female feathering pattern. In two henny-feathered breeds, the Sebright bantam and the Golden Campine, the synthesis of estrogen is increased as a consequence of increased activity of aromatase, a cytochrome P-450 enzyme that converts androgen to estrogen. The activi...

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

authors： Leshin M

更新日期：1985-05-01 00:00:00

abstract：:A human beta-globin gene derived from an established human lymphoblast cell line was introduced into murine erythroleukemia (MEL) cells by cell fusion. The globin genes in MEL cells are inducible by dimethyl sulfoxide (Me2SO); induction leads to the accumulation of mouse globin mRNA and hemoglobin. Globin mRNA was not...

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

authors： Pyati J,Kucherlapati RS,Skoultchi AI

更新日期：1980-06-01 00:00:00

abstract：:The three-dimensional structure of the N-acyl-l-homoserine lactone hydrolase (AHL lactonase) from Bacillus thuringiensis has been determined, by using single-wavelength anomalous dispersion (SAD) phasing, to 1.6-angstroms resolution. AHLs are produced by many Gram-negative bacteria as signaling molecules used in quoru...

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

authors： Liu D,Lepore BW,Petsko GA,Thomas PW,Stone EM,Fast W,Ringe D

更新日期：2005-08-16 00:00:00

abstract：:We show that a large class of finite factors has free entropy dimension less than or equal to one. This class includes all prime factors and many property T factors. ...

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

authors： Ge L,Shen J

更新日期：2000-08-29 00:00:00

abstract：:Using [3H]quinuclidinyl benzilate as a specific cholinergic muscarinic ligand, it has been demonstrated that lymphocytes have muscarinic binding sites. There are approximately 200 sites per cell and the dissociation constant for quinuclidinyl benzilate is approximately 1 X 10(-9) M. Quinuclidinyl benzilate receptor bi...

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

authors： Gordon MA,Cohen JJ,Wilson IB

更新日期：1978-06-01 00:00:00

abstract：:Nascent replicative form type II (RFII) DNA of coliphage M13 synthesized in an Escherichia coli mutant deficient in the 5' leads to 3' exonuclease associated uith DNA polymerase I contains ribonucleotides that are retained in the covalently closed RFI DNA sealed in vitro by the joint action of T5 phage DNA polymerase ...

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

authors： Dasgupta S,Mitra S

更新日期：1978-01-01 00:00:00

abstract：:Nicotinic AChRs (nAChRs) represent a paradigm for ligand-gated ion channels. Despite intensive studies over many years, our understanding of the mechanisms of activation and inhibition for nAChRs is still incomplete. Here, we present molecular dynamics (MD) simulations of the alpha7 nAChR ligand-binding domain, both i...

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

authors： Yi M,Tjong H,Zhou HX

更新日期：2008-06-17 00:00:00

abstract：:There is a gap between how many scientists communicate and how most people understand and interpret messages. This article argues that the extensive science communications literature needs to be joined by the health literacy literature and anthropological work on cultural variations in hearing and understanding messag...

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

authors： Scrimshaw SC

更新日期：2019-04-16 00:00:00