Constitutive achlorhydria in mucolipidosis type IV.

abstract：:During primate evolution, a major factor in lengthening life-span and decreasing age-specific cancer rates may have been improved protective mechanisms against oxygen radicals. We propose that one of these protective systems is plasma uric acid, the level of which increased markedly during primate evolution as a conse...

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

authors： Ames BN,Cathcart R,Schwiers E,Hochstein P

更新日期：1981-11-01 00:00:00

abstract：:Spectral methods are of fundamental importance in statistics and machine learning, because they underlie algorithms from classical principal components analysis to more recent approaches that exploit manifold structure. In most cases, the core technical problem can be reduced to computing a low-rank approximation to a...

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

authors： Belabbas MA,Wolfe PJ

更新日期：2009-01-13 00:00:00

abstract：:The interaction of the NH2-terminal DNA-binding domain of lac repressor with synthetic oligo[d(AT)] was studied by a photo-CIDNP technique (CIDNP is chemically induced dynamic nuclear polarization). Three of the four tyrosines of the NH2-terminal region were found to be accessible to the photosensitive dye. The corres...

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

authors： Buck F,Rüterjans H,Kaptein R,Beyreuther K

更新日期：1980-09-01 00:00:00

abstract：:Pyrrolysine (Pyl), the 22nd natural amino acid and genetically encoded by UAG, becomes attached to its cognate tRNA by pyrrolysyl-tRNA synthetase (PylRS). We have determined three crystal structures of the Methanosarcina mazei PylRS complexed with either AMP-PNP, Pyl-AMP plus pyrophosphate, or the Pyl analogue N-epsil...

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

authors： Kavran JM,Gundllapalli S,O'Donoghue P,Englert M,Söll D,Steitz TA

更新日期：2007-07-03 00:00:00

abstract：:The multifunctional Creb-binding protein (CBP) protein plays a pivotal role in many critical cellular processes. Here we demonstrate that the bromodomain of CBP binds to histone H3 acetylated on lysine 56 (K56Ac) with higher affinity than to its other monoacetylated binding partners. We show that autoacetylation of CB...

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

authors： Das C,Roy S,Namjoshi S,Malarkey CS,Jones DN,Kutateladze TG,Churchill ME,Tyler JK

更新日期：2014-03-25 00:00:00

abstract：:Immune responses can interfere with the effective use of therapeutic proteins to treat genetic deficiencies and have been challenging to manage. To address this problem, we adapted and studied methods of immune tolerance used in canine organ transplantation research to soluble protein therapeutics. A tolerization regi...

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

authors： Kakkis E,Lester T,Yang R,Tanaka C,Anand V,Lemontt J,Peinovich M,Passage M

更新日期：2004-01-20 00:00:00

abstract：:Increased DNA levels in centrally located endosperm nuclei are shown to be related to endosperm development in Zea mays. Mitotic activity sharply decreases in endosperm cells 10-12 days after pollination. At this time nuclear size and DNA content per nucleus (where C = haploid content) sharply increase until peak leve...

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

authors： Kowles RV,Phillips RL

更新日期：1985-10-01 00:00:00

abstract：:Specific antibodies against types I and III collagens and procollagens were used to localize these proteins in cultured human cells. These studies indicate that the same cell makes both proteins. No type III procollagen synthesis was observed in cells from two patients with two patients with the Ehlers-Danlos type IV ...

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

authors： Gay S,Martin GR,Muller PK,Timpl R,Kuhn K

更新日期：1976-11-01 00:00:00

abstract：:Lupus nephritis (LN) is an autoimmune disease that occurs when autoantibodies complex with self-antigen and form immune complexes that accumulate in the glomeruli. These immune complexes initiate an inflammatory response resulting in glomerular injury. LN often concomitantly affects the tubulointerstitial compartment ...

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

authors： Hao W,Rovin BH,Friedman A

更新日期：2014-09-30 00:00:00

abstract：:We describe a single autoregulatory cassette that allows reversible induction of transgene expression in response to tetracycline (tet). This cassette contains all of the necessary components previously described by others on two separate plasmids that are introduced sequentially over a period of months [Gossen, M. & ...

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

authors： Hofmann A,Nolan GP,Blau HM

更新日期：1996-05-28 00:00:00

abstract：:Rat skeletal muscle selenoprotein W cDNA was isolated and sequenced. The isolation strategy involved design of degenerate PCR primers from reverse translation of a partial peptide sequence. A reverse transcription-coupled PCR product from rat muscle mRNA was used to screen a muscle cDNA library prepared from selenium-...

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

authors： Vendeland SC,Beilstein MA,Yeh JY,Ream W,Whanger PD

更新日期：1995-09-12 00:00:00

abstract：:Defined by monoclonal antibody GA733, the GA733-2 antigen is a cell surface 40-kDa glycoprotein associated with human carcinomas of various origins. Molecular clones for the GA733-2 antigen were isolated from a colorectal carcinoma cell line cDNA library using the high-efficiency COS cell expression system. A 1.4-kilo...

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

authors： Szala S,Froehlich M,Scollon M,Kasai Y,Steplewski Z,Koprowski H,Linnenbach AJ

更新日期：1990-05-01 00:00:00

abstract：:Neural crest cell derivatives have been suggested to be involved in thymus development. We established nonlymphoid thymic stromal cell cultures capable of supporting T-cell differentiation. In these nonlymphoid cell cultures, we identified cells with phenotypic and biochemical markers specific for neuronal cells. Neur...

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

authors： Screpanti I,Meco D,Scarpa S,Morrone S,Frati L,Gulino A,Modesti A

更新日期：1992-04-15 00:00:00

abstract：:The sea anemone, Nematostella vectensis, has become an attractive new model organism for comparative genomics and evolutionary developmental biology. Over the last few years, many genes have been isolated and their expression patterns studied to gain insight into their function. More recently, functional tools have be...

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

authors： Renfer E,Amon-Hassenzahl A,Steinmetz PR,Technau U

更新日期：2010-01-05 00:00:00

abstract：:Recent studies with fibroblasts have shown that peptidylhydroxyproline formation begins toward the end of the log phase of growth and is an expression of the activity of the enzyme, collagen proline hydroxylase, which increase in late log-phase cells. This communication reports a two- to fivefold increase in hydroxyla...

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

authors： Comstock JP,Udenfriend S

更新日期：1970-06-01 00:00:00

abstract：:B-DNA becomes unstable under superhelical stress and is able to adopt a wide range of alternative conformations including strand-separated DNA and Z-DNA. Localized sequence-dependent structural transitions are important for the regulation of biological processes such as DNA replication and transcription. To directly p...

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

authors： Oberstrass FC,Fernandes LE,Bryant Z

更新日期：2012-04-17 00:00:00

abstract：:Better understanding myelination of peripheral nerves would benefit patients affected by peripheral neuropathies, including Charcot-Marie-Tooth disease. Little is known about the role the Golgi compartment plays in Schwann cell (SC) functions. Here, we studied the role of Golgi in myelination of peripheral nerves in m...

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

authors： Baba T,Alvarez-Prats A,Kim YJ,Abebe D,Wilson S,Aldworth Z,Stopfer MA,Heuser J,Balla T

更新日期：2020-11-10 00:00:00

abstract：:The mitochondrial trifunctional protein (TFP) catalyzes three reactions in the fatty acid β-oxidation process. Mutations in the two TFP subunits cause mitochondrial trifunctional protein deficiency and acute fatty liver of pregnancy that can lead to death. Here we report a 4.2-Å cryo-electron microscopy α2β2 tetrameri...

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

authors： Liang K,Li N,Wang X,Dai J,Liu P,Wang C,Chen XW,Gao N,Xiao J

更新日期：2018-07-03 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：:A muscarone-binding material has been found in Torpedo electroplax. The material behaves in several ways as one would expect of the binding component of the acetylcholine receptor, especially in its high affinity for muscarone (approximate K = 7 x 10(-6)M), acetylcholine (approximate K = 1.1 x 10(-6)M), curare, and ni...

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

authors： O'Brien RD,Gilmour LP

更新日期：1969-06-01 00:00:00

abstract：:Seawater Mg:Ca and Sr:Ca ratios are biogeochemical parameters reflecting the Earth-ocean-atmosphere dynamic exchange of elements. The ratios' dependence on the environment and organisms' biology facilitates their application in marine sciences. Here, we present a measured single-laboratory dataset, combined with previ...

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

authors： Lebrato M,Garbe-Schönberg D,Müller MN,Blanco-Ameijeiras S,Feely RA,Lorenzoni L,Molinero JC,Bremer K,Jones DOB,Iglesias-Rodriguez D,Greeley D,Lamare MD,Paulmier A,Graco M,Cartes J,Barcelos E Ramos J,de Lara A,Sanchez-Lea

更新日期：2020-09-08 00:00:00

abstract：:Polycyclic aromatic hydrocarbons and related species have been suggested to play a key role in the astrochemical evolution of the interstellar medium, but the formation mechanism of even their simplest building block--the aromatic benzene molecule--has remained elusive for decades. Here we demonstrate in crossed molec...

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

authors： Jones BM,Zhang F,Kaiser RI,Jamal A,Mebel AM,Cordiner MA,Charnley SB

更新日期：2011-01-11 00:00:00

abstract：:Blood cells transplantation is largely replacing bone marrow transplantation because engraftment is more rapid. This accelerated engraftment is thought to be mediated by relatively mature committed hematopoietic progenitor cells. Herein, we have used a modified rhodamine (Rho) staining procedure to identify and purify...

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

authors： Zijlmans JM,Visser JW,Laterveer L,Kleiverda K,Heemskerk DP,Kluin PM,Willemze R,Fibbe WE

更新日期：1998-01-20 00:00:00

abstract：:Fatty acid transport protein 4 (FATP4), a transmembrane protein in the endoplasmic reticulum (ER), is a recently identified negative regulator of the ER-associated retinal pigment epithelium (RPE)65 isomerase necessary for recycling 11-cis-retinal, the light-sensitive chromophore of both rod and cone opsin visual pigm...

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

authors： Li S,Gordon WC,Bazan NG,Jin M

更新日期：2020-12-15 00:00:00

abstract：:The spatial coordination of growth is of central importance for the regulation of plant tissue architecture. Individual layers, such as the epidermis, are clonally propagated and structurally maintained by symmetric cell divisions that are oriented along the plane of the layer. The developmental control of this proces...

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

authors： Enugutti B,Kirchhelle C,Oelschner M,Torres Ruiz RA,Schliebner I,Leister D,Schneitz K

更新日期：2012-09-11 00:00:00

abstract：:Little is known about stem cell biology or the specialized environments or niches believed to control stem cell renewal and differentiation in self-renewing tissues of the body. Functional assays for stem cells are available only for hematopoiesis and spermatogenesis, and the microenvironment, or niche, for hematopoie...

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

authors： Shinohara T,Orwig KE,Avarbock MR,Brinster RL

更新日期：2001-05-22 00:00:00

abstract：:Morphine produces analgesia at opiate receptors expressed in nociceptive circuits. mu, delta, and kappa opiate receptor subtypes are expressed in circuits that can modulate nociception and receive inputs from endogenous opioid neuropeptide ligands. The roles played by each receptor subtype in nociceptive processing in...

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

authors： Sora I,Takahashi N,Funada M,Ujike H,Revay RS,Donovan DM,Miner LL,Uhl GR

更新日期：1997-02-18 00:00:00

abstract：:Cardiolipin is important for bacterial and mitochondrial stability and function. The final step in cardiolipin biosynthesis is catalyzed by cardiolipin synthase and differs mechanistically between prokaryotes and eukaryotes. To study the importance of cardiolipin synthesis for mitochondrial integrity, membrane protein...

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

authors： Serricchio M,Bütikofer P

更新日期：2012-04-17 00:00:00

abstract：:Remarkable progress has come from whole-brain models linking anatomy and function. Paradoxically, it is not clear how a neuronal dynamical system running in the fixed human anatomical connectome can give rise to the rich changes in the functional repertoire associated with human brain function, which is impossible to ...

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

authors： Kringelbach ML,Cruzat J,Cabral J,Knudsen GM,Carhart-Harris R,Whybrow PC,Logothetis NK,Deco G

更新日期：2020-04-28 00:00:00

abstract：:PCSK9 encodes proprotein convertase subtilisin/kexin type 9a (PCSK9), a member of the proteinase K subfamily of subtilases. Missense mutations in PCSK9 cause an autosomal dominant form of hypercholesterolemia in humans, likely due to a gain-of-function mechanism because overexpression of either WT or mutant PCSK9 redu...

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

authors： Rashid S,Curtis DE,Garuti R,Anderson NN,Bashmakov Y,Ho YK,Hammer RE,Moon YA,Horton JD

更新日期：2005-04-12 00:00:00