Gaucher's disease: deficiency of 'acid' -glucosidase and reconstitution of enzyme activity in vitro.

abstract：:A five-helix secondary structural model is proposed for eukaryotic cytoplasmic 5S rRNA. All available sequence data are consistent with this model including those from Chlorella 5S rRNA whose sequence is revised by data included here. Various architectural features of eukaryotic 5S rRNA are summarized in terms of this...

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

authors： Luehrsen KR,Fox GE

更新日期：1981-04-01 00:00:00

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

authors： Fink S,Excoffier L,Heckel G

更新日期：2006-07-18 00:00:00

abstract：:In adult rodents, neurons are continually generated in the subventricular zone of the forebrain, from where they migrate tangentially toward the olfactory bulb, the only known target for these neuronal precursors. Within the main olfactory bulb, they ascend radially into the granule and periglomerular cell layers, whe...

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

authors： Gheusi G,Cremer H,McLean H,Chazal G,Vincent JD,Lledo PM

更新日期：2000-02-15 00:00:00

abstract：:Most HLA haplotypes carry two expressed DR beta-chain genes; in the DR4 haplotype, the polymorphic locus has been called DR beta 1 and the apparently nonpolymorphic locus has been called DR beta 2. We have isolated nearly full-length DR beta-chain cDNA clones representing each of these two loci from a cell line homozy...

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

authors： Curtsinger JM,Hilden JM,Cairns JS,Bach FH

更新日期：1987-01-01 00:00:00

abstract：:The molecular basis of short-day-induced growth cessation and dormancy in the meristems of perennial plants (e.g., forest trees growing in temperate and high-latitude regions) is poorly understood. Using global transcript profiling, we show distinct stage-specific alterations in auxin responsiveness of the transcripto...

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

authors： Baba K,Karlberg A,Schmidt J,Schrader J,Hvidsten TR,Bako L,Bhalerao RP

更新日期：2011-02-22 00:00:00

abstract：:Despite a growing number of ion channel genes implicated in hereditary ataxia, it remains unclear how ion channel mutations lead to loss-of-function or death of cerebellar neurons. Mutations in the gene KCNMA1, encoding the α-subunit of the BK channel have emerged as responsible for a variety of neurological phenotype...

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

authors： Du X,Carvalho-de-Souza JL,Wei C,Carrasquel-Ursulaez W,Lorenzo Y,Gonzalez N,Kubota T,Staisch J,Hain T,Petrossian N,Xu M,Latorre R,Bezanilla F,Gomez CM

更新日期：2020-03-17 00:00:00

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

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

abstract：:A complete experimental format is given for the reconstitution of human hemoglobin from the separated heme-free alpha- and beta-globin chains (alpha degrees, beta degrees) and hemin, by two alternative routes. Based on their oxygen binding properties, the reaction of the ferri-forms with reducing agent, and the respon...

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

authors： Yip YK,Waks M,Beychok S

更新日期：1977-01-01 00:00:00

abstract：:Fetal exposure to environmental insults increases the susceptibility to late-onset neuropsychiatric disorders. Alcohol is listed as one of such prenatal environmental risk factors and known to exert devastating teratogenetic effects on the developing brain, leading to complex neurological and psychiatric symptoms obse...

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

authors： Hashimoto-Torii K,Kawasawa YI,Kuhn A,Rakic P

更新日期：2011-03-08 00:00:00

abstract：:Genetic manipulation of nitrogenase and key glutamate-forming enzymes can provide mutants that excrete fixed N2 as NH4+. A derepressed N2 fxation mutant (SK-24) has been isolated , which excretes up to 20.2 mumol of fixed N2 as NH4+ per mg of cell protein in 24 hr at room temperature. Biochemical analysis shows that t...

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

authors： Shanmugam KT,Valentine RC

更新日期：1975-01-01 00:00:00

abstract：:Artificially aminoacylated suppressor tRNAs were used to introduce photoreactive amino acids into model mitochondrial precursor proteins to probe the environment along the protein import pathway. Amino acids with benzophenone side chains of various lengths [DL-2-amino-3-(p-benzoylphenyl)propanoic acid (1) and DL-2-ami...

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

authors： Kanamori T,Nishikawa S,Shin I,Schultz PG,Endo T

更新日期：1997-01-21 00:00:00

abstract：:The development of cardiac hypertrophy is mediated, in part, by increase in NADPH oxidase activity and myocardial oxidative stress. The Rho GTPase, Rac, regulates NADPH oxidase activity through interaction with gp91(phox) and p67(phox) (in which "phox" is phagocyte oxidase). However, it is not known which Rac isoform ...

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

authors： Satoh M,Ogita H,Takeshita K,Mukai Y,Kwiatkowski DJ,Liao JK

更新日期：2006-05-09 00:00:00

abstract：:The role played by oligodendrocytes (OLs), the myelinating cells of the CNS, during brain development has not been fully explored. We have addressed this question by inducing a temporal and reversible ablation of OLs on postnatal CNS development. OL ablation in newborn mice leads to a profound alteration in the struct...

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

authors： Collin L,Usiello A,Erbs E,Mathis C,Borrelli E

更新日期：2004-01-06 00:00:00

abstract：:We describe a model for early onset preeclampsia (EOPE) that uses induced pluripotent stem cells (iPSCs) generated from umbilical cords of EOPE and control (CTL) pregnancies. These iPSCs were then converted to placental trophoblast (TB) representative of early pregnancy. Marker gene analysis indicated that both sets o...

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

authors： Sheridan MA,Yang Y,Jain A,Lyons AS,Yang P,Brahmasani SR,Dai A,Tian Y,Ellersieck MR,Tuteja G,Schust DJ,Schulz LC,Ezashi T,Roberts RM

更新日期：2019-03-05 00:00:00

abstract：:The IgG2a monoclonal antibody TH-1, which reacts specifically with blood group A1 but with neither A2 nor O erythrocytes, has been established. The antibody reacted only with A1 erythrocytes in hemagglutination and antibody absorption assays; it did not react with A2 erythrocytes, even after trypsin or sialidase treat...

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

authors： Clausen H,Levery SB,Nudelman E,Tsuchiya S,Hakomori S

更新日期：1985-02-01 00:00:00

abstract：:Unit-resolution mass spectra have been obtained for peptides as large as 17 kDa, providing information on impurities and adduct ions, as well as accurate molecular weight values. Electrospray ionization produces many multiply-charged species of the same mass; isotopic peak resolution provides direct charge state assig...

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

authors： Loo JA,Quinn JP,Ryu SI,Henry KD,Senko MW,McLafferty FW

更新日期：1992-01-01 00:00:00

abstract：:Dynamic signals linking the actin cytoskeleton and cell adhesion receptors are essential for morphogenesis during development and normal tissue homeostasis. Abi1 is a central regulator of actin polymerization through interactions with multiple protein complexes. However, the in vivo role of Abi1 remains to be defined....

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

authors： Ring C,Ginsberg MH,Haling J,Pendergast AM

更新日期：2011-01-04 00:00:00

abstract：:The [A] allele of SNP rs965513 in 9q22 has been consistently shown to be highly associated with increased papillary thyroid cancer (PTC) risk with an odds ratio of ∼1.8 as determined by genome-wide association studies, yet the molecular mechanisms remain poorly understood. Previously, we noted that the expression of t...

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

authors： He H,Li W,Liyanarachchi S,Srinivas M,Wang Y,Akagi K,Wang Y,Wu D,Wang Q,Jin V,Symer DE,Shen R,Phay J,Nagy R,de la Chapelle A

更新日期：2015-05-12 00:00:00

abstract：:Explanations for primate brain expansion and the evolution of human cognition and culture remain contentious despite extensive research. While multiple comparative analyses have investigated variation in brain size across primate species, very few have addressed why primates vary in how much they use social learning. ...

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

authors： Street SE,Navarrete AF,Reader SM,Laland KN

更新日期：2017-07-25 00:00:00

abstract：:A convenient technique for the partial purification of large quantities of functional, poly(adenylic acid)-rich mRNA is described. The method depends upon annealing poly(adenylic acid)-rich mRNA to oligothymidylic acid-cellulose columns and its elution with buffers of low ionic strength. Biologically active rabbit glo...

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

authors： Aviv H,Leder P

更新日期：1972-06-01 00:00:00

abstract：:Bone remodeling requires osteoclasts to generate and maintain an acidified resorption compartment between the apical membrane and the bone surface to solubilize hydroxyapatite crystals within the bone matrix. This acidification process requires (i) apical proton secretion by a vacuolar H(+)-ATPase, (ii) actin cytoskel...

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

authors： Coury F,Zenger S,Stewart AK,Stephens S,Neff L,Tsang K,Shull GE,Alper SL,Baron R,Aliprantis AO

更新日期：2013-02-05 00:00:00

abstract：:The density of corn pollen on leaves of milkweed plants inside and outside of cornfields was measured in several studies from different localities. The purpose was to obtain a representative picture of naturally occurring pollen densities to provide a perspective for laboratory and field studies of monarch larvae feed...

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

authors： Pleasants JM,Hellmich RL,Dively GP,Sears MK,Stanley-Horn DE,Mattila HR,Foster JE,Clark P,Jones GD

更新日期：2001-10-09 00:00:00

abstract：:One key to the success of Mycobacterium tuberculosis as a pathogen is its ability to reside in the hostile environment of the human macrophage. Bacteria adapt to stress through a variety of mechanisms, including the use of small regulatory RNAs (sRNAs), which posttranscriptionally regulate bacterial gene expression. H...

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

authors： Gerrick ER,Barbier T,Chase MR,Xu R,François J,Lin VH,Szucs MJ,Rock JM,Ahmad R,Tjaden B,Livny J,Fortune SM

更新日期：2018-06-19 00:00:00

abstract：:There is increasing, but largely indirect, evidence pointing to an effect of commensal gut microbiota on the central nervous system (CNS). However, it is unknown whether lactic acid bacteria such as Lactobacillus rhamnosus could have a direct effect on neurotransmitter receptors in the CNS in normal, healthy animals. ...

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

authors： Bravo JA,Forsythe P,Chew MV,Escaravage E,Savignac HM,Dinan TG,Bienenstock J,Cryan JF

更新日期：2011-09-20 00:00:00

abstract：:The possibility that glycolate oxidation in unicellular green algae is linked to mitochondrial electron transport, rather than to peroxisomal metabolism as in higher plants and animals, was studied in a mutant of Chlamydomonas reinhardtii (dk97) deficient in cytochrome oxidase. This mutant had normal rates of dark res...

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

authors： Husic DW,Tolbert NE

更新日期：1987-03-01 00:00:00

abstract：:The E3 ubiquitin ligase X-linked inhibitor of apoptosis (XIAP) acts as a molecular rheostat for the immune deficiency (IMD) pathway of the tick Ixodes scapularis How XIAP activates the IMD pathway in response to microbial infection remains ill defined. Here, we identified the XIAP enzymatic substrate p47 as a positive...

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

authors： McClure Carroll EE,Wang X,Shaw DK,O'Neal AJ,Oliva Chávez AS,Brown LJ,Boradia VM,Hammond HL,Pedra JHF

更新日期：2019-01-02 00:00:00

abstract：:Apolipoprotein A-I (apo A-I), the major apolipoprotein in human high density lipoproteins, is involved in the disease atherosclerosis. Cloned apo A-I cDNA (pA1-3) was used as a probe in chromosome mapping studies to detect the human apo A-I structural gene sequence in human-Chinese hamster cell hybrids. Southern blot ...

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

authors： Cheung P,Kao FT,Law ML,Jones C,Puck TT,Chan L

更新日期：1984-01-01 00:00:00

abstract：:A series of HIV integrase (HIV-1 IN) inhibitors were synthesized to evaluate the role of the metal-binding group (MBG) in this class of metalloenzyme inhibitors. A total of 21 different raltegravir-chelator derivative (RCD) compounds were prepared that differed only in the nature of the MBG. These IN strand-transfer i...

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

authors： Agrawal A,DeSoto J,Fullagar JL,Maddali K,Rostami S,Richman DD,Pommier Y,Cohen SM

更新日期：2012-02-14 00:00:00

abstract：:Hereditary hemochromatosis (HH) is a disorder of iron metabolism caused by common mutations in the gene HFE. The HFE protein binds to transferrin receptor-1 (TfR1) in competition with transferrin, and in vitro, reduces cellular iron by reducing iron uptake. However, in vivo, HFE is strongly expressed by liver macropha...

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

authors： Drakesmith H,Sweetland E,Schimanski L,Edwards J,Cowley D,Ashraf M,Bastin J,Townsend AR

更新日期：2002-11-26 00:00:00

abstract：:The DNA damage response is an essential process for the survival of living cells. In a subset of stress-responsive genes in humans, Elongin controls transcription in response to multiple stimuli, such as DNA damage, oxidative stress, and heat shock. Yeast Elongin (Ela1-Elc1), along with Def1, is known to facilitate ub...

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

authors： Damodaren N,Van Eeuwen T,Zamel J,Lin-Shiao E,Kalisman N,Murakami K

更新日期：2017-12-12 00:00:00