Tangier disease: a disorder of intracellular membrane traffic.

abstract：:In skeletal muscle, dihydropyridine receptors and dihydropyridine-sensitive Ca2+ channels are preferentially localized in the transverse tubular membranes. Starting with an antigenic membrane fraction enriched in rabbit muscle transverse tubules (T-tubules), several monoclonal antibodies were produced by a fusion of s...

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

authors： Malouf NN,Coronado R,McMahon D,Meissner G,Gillespie GY

更新日期：1987-07-01 00:00:00

abstract：:Genotype-phenotype relationships are notoriously complicated. Idiosyncratic interactions between specific combinations of mutations occur and are difficult to predict. Yet it is increasingly clear that many interactions can be understood in terms of global epistasis. That is, mutations may act additively on some under...

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

authors： Otwinowski J,McCandlish DM,Plotkin JB

更新日期：2018-08-07 00:00:00

abstract：:Degradation of the most abundant membrane protein on earth, the light-harvesting complex of Photosystem II (LHC II), is highly regulated under various environmental conditions, e.g., light stress, to prevent photochemical damage to the reaction center. We identified the LHC II degrading protease in Arabidopsis thalian...

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

authors： Zelisko A,García-Lorenzo M,Jackowski G,Jansson S,Funk C

更新日期：2005-09-20 00:00:00

abstract：:The functional significance of the portal vascular link between the beta cells of the pancreatic islets and the liver has not been established. Previous studies indicated that insulin does not acutely regulate glucose metabolism by a direct hepatic effect. More recent observations suggest that the role of insulin in r...

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

authors： Griffen SC,Russell SM,Katz LS,Nicoll CS

更新日期：1987-10-01 00:00:00

abstract：:The Drosophila melanogaster urate oxidase (UO)-encoding gene is expressed in the third-instar larva and adult. In contrast, the Drosophila pseudoobscura UO gene is only expressed in the adult, whereas the Drosophila virilis UO gene is expressed only in the third-instar larva. UO activity in these three Drosophila spec...

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

authors： Wallrath LL,Friedman TB

更新日期：1991-07-01 00:00:00

abstract：:Sex is thought to facilitate accumulation of initially rare beneficial mutations by allowing simultaneous allele replacements at many loci. However, this advantage of sex depends on a restrictive assumption that the fitness of a genotype is determined by fitness potential, a single intermediate variable to which all l...

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

authors： Kondrashov FA,Kondrashov AS

更新日期：2001-10-09 00:00:00

abstract：:Native cytochrome c (cyt c) has a compact tertiary structure with a hexacoordinated heme iron and functions in electron transport in mitochondria and apoptosis in the cytoplasm. However, the possibility that protein modifications confer additional functions to cyt c has not been explored. Disruption of methionine 80 (...

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

authors： Godoy LC,Muñoz-Pinedo C,Castro L,Cardaci S,Schonhoff CM,King M,Tórtora V,Marín M,Miao Q,Jiang JF,Kapralov A,Jemmerson R,Silkstone GG,Patel JN,Evans JE,Wilson MT,Green DR,Kagan VE,Radi R,Mannick JB

更新日期：2009-02-24 00:00:00

abstract：:Groundwater-dependent species are among the least-known components of global biodiversity, as well as some of the most vulnerable because of rapid groundwater depletion at regional and global scales. The karstic Edwards-Trinity aquifer system of west-central Texas is one of the most species-rich groundwater systems in...

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

authors： Devitt TJ,Wright AM,Cannatella DC,Hillis DM

更新日期：2019-02-12 00:00:00

abstract：:A theory is proposed to study the sensitivity of cylindrical explosives. Using the equations governing the conservation of mass, energy, and momentum, the equation of state, and Newtonian mechanics, we have derived the expression D = D(o)e(-a2/d), in which the apparent length of the reaction zone, a, takes into accoun...

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

authors： Eyring H,Walker FE,Ma SM,Coon N

更新日期：1980-05-01 00:00:00

abstract：:Femtosecond and subfemtosecond time scales typically rule electron dynamics at metal surfaces. Recent advance in experimental techniques permits now remarkable precision in the description of these processes. In particular, shorter time scales, smaller system sizes, and spin-dependent effects are current targets of in...

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

authors： Muiño RD,Sánchez-Portal D,Silkin VM,Chulkov EV,Echenique PM

更新日期：2011-01-18 00:00:00

abstract：:Dysfunction of gap junctions (GJs) caused by mutations in connexin26 (Cx26) and Cx30 accounts for nearly half of all cases of hereditary nonsyndromic deafness cases. Although it is widely held that GJs connecting supporting cells in the organ of Corti mainly provide ionic pathways for rapid removal of K+ around the ba...

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

authors： Zhang Y,Tang W,Ahmad S,Sipp JA,Chen P,Lin X

更新日期：2005-10-18 00:00:00

abstract：:Serotonin (5-HT) plays a critical role in modulating synaptic plasticity in the marine mollusc Aplysia and in the mammalian nervous system. In Aplysia sensory neurons, 5-HT can activate several signal cascades, including PKA and PKC, presumably via distinct types of G protein-coupled receptors. However, the molecular ...

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

authors： Lee YS,Choi SL,Lee SH,Kim H,Park H,Lee N,Lee SH,Chae YS,Jang DJ,Kandel ER,Kaang BK

更新日期：2009-08-25 00:00:00

abstract：:It is found that the addition of hexamethylphosphoramide to the solution of an alkyne, Et(2)Zn, and (S)-1,1'-bi-2-naphthol in methylene chloride allows the generation of an alkynylzinc at room temperature and shows highly enantioselective additions to aldehydes. The mild condition for the formation of the alkynylzinc ...

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

authors： Gao G,Xie RG,Pu L

更新日期：2004-04-13 00:00:00

abstract：:The origin of plant chemical ecology generally dates to the late 1950s, when evolutionary entomologists recognized the essential role of plant secondary metabolites in plant-insect interactions and suggested that plant chemical diversity evolved under the selection pressure of herbivory. However, similar ideas had alr...

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

authors： Hartmann T

更新日期：2008-03-25 00:00:00

abstract：:We have compared the behavior in solution of the synthetic polynucleotide poly(dG-m5dC).poly(dG-m5dC) with that of the unmethylated polynucleotide poly(dG-dC).poly(dG-dC). In solutions containing high concentrations of salt, poly(dG-dC).poly(dG-dC) is known to exhibit altered circular dichroic and absorption spectra c...

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

authors： Behe M,Felsenfeld G

更新日期：1981-03-01 00:00:00

abstract：:Helper T lymphocytes recognize fragments of foreign (or self) antigens in the peptide-binding clefts of major histocompatibility complex class II molecules; their activation is a crucial step in the induction of many immune and autoimmune responses. While studying the latter, we raised a T-cell line from the thymus of...

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

authors： Ong B,Willcox N,Wordsworth P,Beeson D,Vincent A,Altmann D,Lanchbury JS,Harcourt GC,Bell JI,Newsom-Davis J

更新日期：1991-08-15 00:00:00

abstract：:Ral (Ras-like) GTPases are directly activated by oncogenic Ras GTPases. Mutant K-Ras (G12C) has enabled the development of covalent K-Ras inhibitors currently in clinical trials. However, Ral, and the overwhelming majority of mutant oncogenic K-Ras, are devoid of a druggable pocket and lack an accessible cysteine for ...

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

authors： Bum-Erdene K,Liu D,Gonzalez-Gutierrez G,Ghozayel MK,Xu D,Meroueh SO

更新日期：2020-03-31 00:00:00

abstract：:Noncompetitive inhibitors of the nicotinic acetylcholine (ACh) receptors suppress cation flux directly by binding in and blocking the open channel or indirectly by stabilizing closed states of the receptor. The lidocaine derivative QX-314 and the acridine derivative quinacrine act directly as open channel blockers, bu...

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

authors： Yu Y,Shi L,Karlin A

更新日期：2003-04-01 00:00:00

abstract：:Lactobacillus acidophilus NCFM is a probiotic bacterium that has been produced commercially since 1972. The complete genome is 1,993,564 nt and devoid of plasmids. The average GC content is 34.71% with 1,864 predicted ORFs, of which 72.5% were functionally classified. Nine phage-related integrases were predicted, but ...

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

authors： Altermann E,Russell WM,Azcarate-Peril MA,Barrangou R,Buck BL,McAuliffe O,Souther N,Dobson A,Duong T,Callanan M,Lick S,Hamrick A,Cano R,Klaenhammer TR

更新日期：2005-03-15 00:00:00

abstract：:The combination of catalytic amounts of optically active dipyridylphosphine and CuF(2) along with hydride donor PhSiH(3) generated in situ a remarkably reactive catalyst system (substrate-to-ligand molar ratio up to 100,000) for the highly enantioselective hydrosilylation of a broad spectrum of aryl alkyl ketones (up ...

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

authors： Wu J,Ji JX,Chan AS

更新日期：2005-03-08 00:00:00

abstract：:Visual development is thought to be completed at an early age. We suggest that the maturation of the visual brain is not homogeneous: functions with greater need for early availability, such as visuomotor control, mature earlier, and the development of other visual functions may extend well into childhood. We found si...

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

authors： Kovács I,Kozma P,Fehér A,Benedek G

更新日期：1999-10-12 00:00:00

abstract：:Bone mineralization is an essential step during the embryonic development of vertebrates, and bone serves vital functions in human physiology. To systematically identify unique gene functions essential for osteogenesis, we performed a forward genetic screen in zebrafish and isolated a mutant, no bone (nob), that does ...

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

authors： Huitema LF,Apschner A,Logister I,Spoorendonk KM,Bussmann J,Hammond CL,Schulte-Merker S

更新日期：2012-12-26 00:00:00

abstract：:Accumulating evidence suggests that subcutaneous and visceral adipose tissues are differentially associated with metabolic disorders. In obesity, subcutaneous adipose tissue is beneficial for metabolic homeostasis because of repressed inflammation. However, the underlying mechanism remains unclear. Here, we demonstrat...

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

authors： Hwang I,Jo K,Shin KC,Kim JI,Ji Y,Park YJ,Park J,Jeon YG,Ka S,Suk S,Noh HL,Choe SS,Alfadda AA,Kim JK,Kim S,Kim JB

更新日期：2019-06-11 00:00:00

abstract：:Calcium oscillations suppress mitochondrial movements along the microtubules to support on-demand distribution of mitochondria. To activate this mechanism, Ca(2+) targets a yet unidentified cytoplasmic factor that does not seem to be a microtubular motor or a kinase/phosphatase. Here, we have studied the dependence of...

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

authors： Saotome M,Safiulina D,Szabadkai G,Das S,Fransson A,Aspenstrom P,Rizzuto R,Hajnóczky G

更新日期：2008-12-30 00:00:00

abstract：:The exceptional sensitivity of Mycobacterium tuberculosis to isonicotinic acid hydrazide (INH) lacks satisfactory definition. M. tuberculosis is a natural mutant in oxyR, a central regulator of peroxide stress response. The ahpC gene, which encodes a critical subunit of alkyl hydroperoxide reductase, is one of the tar...

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

authors： Zhang Y,Dhandayuthapani S,Deretic V

更新日期：1996-11-12 00:00:00

abstract：:Studies of tumors from human familial adenomatous polyposis, sporadic colon cancer, and mouse and rat models of intestinal cancer indicate that the majority of early adenomas develop through loss of normal function of the Adenomatous polyposis coli (APC) gene. In murine models of familial adenomatous polyposis, specif...

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

authors： Amos-Landgraf JM,Irving AA,Hartman C,Hunter A,Laube B,Chen X,Clipson L,Newton MA,Dove WF

更新日期：2012-02-07 00:00:00

abstract：:Photosystem I (PSI) reaction centers (RCs) of the thermophilic cyanobacterium Mastigocladus laminosus were purified and characterized. The PSI RC was obtained in two forms, monomeric and trimeric. The two forms contained the same number of pigments per P700 and displayed similar photochemical activities. The two forms...

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

authors： Almog O,Shoham G,Michaeli D,Nechushtai R

更新日期：1991-06-15 00:00:00

abstract：:Molecular mechanisms that initiate meiosis have been studied in fungi and mammals, but little is known about the mechanisms directing the meiosis transition in other organisms. To elucidate meiosis initiation in plants, we characterized and cloned the ameiotic1 (am1) gene, which affects the transition to meiosis and p...

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

authors： Pawlowski WP,Wang CJ,Golubovskaya IN,Szymaniak JM,Shi L,Hamant O,Zhu T,Harper L,Sheridan WF,Cande WZ

更新日期：2009-03-03 00:00:00

abstract：:A key barrier to the realization of personalized medicine for cancer is the identification of biomarkers. Here we describe a two-stage strategy for the discovery of serum biomarker signatures corresponding to specific cancer-causing mutations and its application to prostate cancer (PCa) in the context of the commonly ...

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

authors： Cima I,Schiess R,Wild P,Kaelin M,Schüffler P,Lange V,Picotti P,Ossola R,Templeton A,Schubert O,Fuchs T,Leippold T,Wyler S,Zehetner J,Jochum W,Buhmann J,Cerny T,Moch H,Gillessen S,Aebersold R,Krek W

更新日期：2011-02-22 00:00:00

abstract：:The effects of phorbol 12-myristate 13-acetate (PMA), a potent activator of protein kinase C, on Na+ influx were investigated in cultured human foreskin fibroblasts (HSWP cells). We report here that in serum-deprived HSWP cells the addition of PMA alone has no significant effect on Na+ influx. However, the addition of...

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

authors： Vicentini LM,Villereal ML

更新日期：1985-12-01 00:00:00