当前位置： SCI文献检索 > CELL期刊下所有文献 > Expression of cDNA encoding the human "protective protein" associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases.

Expression of cDNA encoding the human "protective protein" associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases.

Abstract：

:The "protective protein" is a glycoprotein that associates with lysosomal beta-galactosidase and neuraminidase and is deficient in the autosomal recessive disorder galactosialidosis. We have isolated the cDNA encoding human "protective protein". The clone recognizes a 2 kb mRNA in normal cells that is not evident in fibroblasts of an early infantile galactosialidosis patient. The cDNA directs the synthesis of a 452 amino acid precursor molecule that is processed in vivo to yield mature "protective protein," a heterodimer of 32 kd and 20 kd polypeptides held together by disulfide bridges. This mature form is also biologically functional since it restores beta-galactosidase and neuraminidase activities in galactosialidosis cells. The predicted amino acid sequence of the "protective protein" bears homology to yeast carboxypeptidase Y and the KEX1 gene product. This suggests a protease activity for the "protective protein."

journal_name

Cell

journal_title

Cell

authors

Galjart NJ,Gillemans N,Harris A,van der Horst GT,Verheijen FW,Galjaard H,d'Azzo A

doi

10.1016/s0092-8674(88)90999-3

subject

Has Abstract

pub_date

1988-09-09 00:00:00

pages

755-64

issue

6

eissn

0092-8674

issn

1097-4172

pii

S0092-8674(88)90999-3

journal_volume

54

pub_type

杂志文章

相关文献

CELL文献大全
  • In yeast, RAS proteins are controlling elements of adenylate cyclase.

    abstract：:S. cerevisiae strains containing RAS2val19, a RAS2 gene with a missense mutation analogous to one that activates the transforming potential of mammalian ras genes, have growth and biochemical properties strikingly similar to yeast strains carrying IAC or bcy1. Yeast strains carrying the IAC mutation have elevated leve...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(85)90305-8

    authors： Toda T,Uno I,Ishikawa T,Powers S,Kataoka T,Broek D,Cameron S,Broach J,Matsumoto K,Wigler M

    更新日期：1985-01-01 00:00:00

  • Proteolysis-Targeting Chimeras as Therapeutics and Tools for Biological Discovery.

    abstract：:New biological tools provide new techniques to probe fundamental biological processes. Here we describe the burgeoning field of proteolysis-targeting chimeras (PROTACs), which are capable of modulating protein concentrations at a post-translational level by co-opting the ubiquitin-proteasome system. We describe the PR...

    journal_title：Cell

    pub_type： 杂志文章,评审

    doi：10.1016/j.cell.2019.11.031

    authors： Burslem GM,Crews CM

    更新日期：2020-04-02 00:00:00

  • Three-dimensional structure of an evolutionarily conserved N-terminal domain of syntaxin 1A.

    abstract：:Syntaxin 1A plays a central role in neurotransmitter release through multiple protein-protein interactions. We have used NMR spectroscopy to identify an autonomously folded N-terminal domain in syntaxin 1A and to elucidate its three-dimensional structure. This 120-residue N-terminal domain is conserved in plasma membr...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/s0092-8674(00)81742-0

    authors： Fernandez I,Ubach J,Dulubova I,Zhang X,Südhof TC,Rizo J

    更新日期：1998-09-18 00:00:00

  • A SWI/SNF-related chromatin remodeling complex, E-RC1, is required for tissue-specific transcriptional regulation by EKLF in vitro.

    abstract：:Erythroid Krüppel-like factor (EKLF) is necessary for stage-specific expression of the human beta-globin gene. We show that EKLF requires a SWI/SNF-related chromatin remodeling complex, EKLF coactivator-remodeling complex 1 (E-RC1), to generate a DNase I hypersensitive, transcriptionally active beta-globin promoter on...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/s0092-8674(00)81785-7

    authors： Armstrong JA,Bieker JJ,Emerson BM

    更新日期：1998-10-02 00:00:00

  • Immediate glycosylation of Sindbis virus membrane proteins.

    abstract：:The mechanism by which the membrane proteins of Sindbis virus are initially glycosylated during growth of the virus in chick cells was studied. The experiments suggest strongly that the two viral glycoproteins are glycosylated before release from the polysome, and that this glycosylation involves transfer of a large 1...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(77)90099-x

    authors： Sefton BM

    更新日期：1977-04-01 00:00:00

  • Structural convergence between Cryo-EM and NMR reveals intersubunit interactions critical for HIV-1 capsid function.

    abstract：:Mature HIV-1 particles contain conical-shaped capsids that enclose the viral RNA genome and perform essential functions in the virus life cycle. Previous structural analysis of two- and three-dimensional arrays of the capsid protein (CA) hexamer revealed three interfaces. Here, we present a cryoEM study of a tubular a...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2009.10.010

    authors： Byeon IJ,Meng X,Jung J,Zhao G,Yang R,Ahn J,Shi J,Concel J,Aiken C,Zhang P,Gronenborn AM

    更新日期：2009-11-13 00:00:00

  • Migratory Neural Crest Cells Phagocytose Dead Cells in the Developing Nervous System.

    abstract：:During neural tube closure and spinal cord development, many cells die in both the central and peripheral nervous systems (CNS and PNS, respectively). However, myeloid-derived professional phagocytes have not yet colonized the trunk region during early neurogenesis. How apoptotic cells are removed from this region dur...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2019.08.001

    authors： Zhu Y,Crowley SC,Latimer AJ,Lewis GM,Nash R,Kucenas S

    更新日期：2019-09-19 00:00:00

  • Synthesis of hybrid bacterial plasmids containing highly repeated satellite DNA.

    abstract：:Hybrid plasmid molecules containing tandemly repeated Drosophila satellite DNA were constructed using a modification of the (dA)-(dT) homopolymer procedure of Lobban and Kaiser (1973). Recombinant plasmids recovered after transformation of recA bacteria contained 10% of the amount of satellite DNA present in the trans...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(77)90038-1

    authors： Brutlag D,Fry K,Nelson T,Hung P

    更新日期：1977-03-01 00:00:00

  • Each of the activities of signal recognition particle (SRP) is contained within a distinct domain: analysis of biochemical mutants of SRP.

    abstract：:Signal recognition particle (SRP), a small ribonucleoprotein required for targeting secretory proteins to the ER, has three known functions: signal recognition, elongation arrest, and translocation promotion. Because SRP is inactivated by the sulfhydryl alkylating reagent N-ethylmaleimide (NEM), we have attempted to e...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(88)90529-6

    authors： Siegel V,Walter P

    更新日期：1988-01-15 00:00:00

  • Mnd2, an essential antagonist of the anaphase-promoting complex during meiotic prophase.

    abstract：:Meiotic cohesin serves in sister chromatid linkage and DNA repair until its subunit Rec8 is cleaved by separase. Separase is activated when its inhibitor, securin, is polyubiquitinated by the Cdc20 regulated anaphase-promoting complex (APC(Cdc20)) and consequently degraded. Differently regulated APCs (APC(Cdh1), APC(A...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2005.01.017

    authors： Penkner AM,Prinz S,Ferscha S,Klein F

    更新日期：2005-03-25 00:00:00

  • The hows and Ys of genome integrity.

    abstract：:Much of the Y chromosome consists of large palindromic arrays harboring genes that are critical for spermatogenesis. In this issue, Lange et al. (2009) show that although gene conversion within these arrays maintains their integrity, it also permits rare unequal sister chromatid-exchange events within palindromes that...

    journal_title：Cell

    pub_type： 评论,杂志文章

    doi：10.1016/j.cell.2009.08.022

    authors： Hall HE,Hawley RS

    更新日期：2009-09-04 00:00:00

  • p53 opens the mitochondrial permeability transition pore to trigger necrosis.

    abstract：:Ischemia-associated oxidative damage leading to necrosis is a major cause of catastrophic tissue loss, and elucidating its signaling mechanism is therefore of paramount importance. p53 is a central stress sensor responding to multiple insults, including oxidative stress to orchestrate apoptotic and autophagic cell dea...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2012.05.014

    authors： Vaseva AV,Marchenko ND,Ji K,Tsirka SE,Holzmann S,Moll UM

    更新日期：2012-06-22 00:00:00

  • Primary structure and transcriptional regulation of GAP-43, a protein associated with nerve growth.

    abstract：:Nerve regeneration and developmental outgrowth of axons are both correlated with increased synthesis of an axonal membrane protein designated GAP-43. Phosphorylation of an apparently identical protein, present at lower abundance in adult brains, has been correlated with long-term potentiation, a form of synaptic plast...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(87)90616-7

    authors： Basi GS,Jacobson RD,Virág I,Schilling J,Skene JH

    更新日期：1987-06-19 00:00:00

  • New evidence supporting megakaryocyte-erythrocyte potential of flk2/flt3+ multipotent hematopoietic progenitors.

    abstract：:A model of hematopoietic development wherein multipotentiality is conserved until segregation of myeloid and lymphoid potential has recently been challenged, proposing that megakaryocyte/erythrocyte (MegE) potential is lost in Flk2/Flt3-expressing early progenitors. Here, we used sensitive in vivo approaches to quanti...

    journal_title：Cell

    pub_type： 信件

    doi：10.1016/j.cell.2006.06.037

    authors： Forsberg EC,Serwold T,Kogan S,Weissman IL,Passegué E

    更新日期：2006-07-28 00:00:00

  • Secondary structure of mouse and rabbit alpha- and beta-globin mRNAs: differential accessibility of alpha and beta initiator AUG codons towards nucleases.

    abstract：:The nucleotide sequence from the 5' terminus inward of one third of mouse alpha- and beta maj-globin messenger RNAs has been established. In addition, using 5' 32P end-labeled mRNAs as substrates and S1 and T1 nucleases as probes for single-stranded regions, the secondary structures of mouse and rabbit alpha- and beta...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(80)90391-8

    authors： Pavlakis GN,Lockard RE,Vamvakopoulos N,Rieser L,RajBhandary UL,Vournakis JN

    更新日期：1980-01-01 00:00:00

  • Disruption of the Hoxd-13 gene induces localized heterochrony leading to mice with neotenic limbs.

    abstract：:Vertebrate Hoxd genes are sequentially activated during the morphogenesis and pattern formation of the limb. Using the approach of gene disruption via homologous recombination in embryonic stem cells, we have assessed the function of the last gene of the complex, Hoxd-13. Mutant mice displayed skeletal alterations alo...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(93)90378-4

    authors： Dollé P,Dierich A,LeMeur M,Schimmang T,Schuhbaur B,Chambon P,Duboule D

    更新日期：1993-11-05 00:00:00

  • Intercellular nanotubes mediate bacterial communication.

    abstract：:Bacteria are known to communicate primarily via secreted extracellular factors. Here we identify a previously uncharacterized type of bacterial communication mediated by nanotubes that bridge neighboring cells. Using Bacillus subtilis as a model organism, we visualized transfer of cytoplasmic fluorescent molecules bet...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2011.01.015

    authors： Dubey GP,Ben-Yehuda S

    更新日期：2011-02-18 00:00:00

  • Common Features of Enveloped Viruses and Implications for Immunogen Design for Next-Generation Vaccines.

    abstract：:Enveloped viruses enter cells by inducing fusion of viral and cellular membranes, a process catalyzed by a specialized membrane-fusion protein expressed on their surface. This review focuses on recent structural studies of viral fusion proteins with an emphasis on their metastable prefusion form and on interactions wi...

    journal_title：Cell

    pub_type： 杂志文章,评审

    doi：10.1016/j.cell.2018.02.054

    authors： Rey FA,Lok SM

    更新日期：2018-03-08 00:00:00

  • The discovery of statins.

    abstract：:This year, the Lasker Foundation confers its Clinical Medical Research Award on Akira Endo for his isolation from fungi of statins, potent inhibitors of cholesterol synthesis in the liver. The introduction of statins to clinical practice has markedly reduced morbidity and mortality from atherosclerotic cardiovascular ...

    journal_title：Cell

    pub_type： 历史文章,杂志文章

    doi：10.1016/j.cell.2008.09.008

    authors： Stossel TP

    更新日期：2008-09-19 00:00:00

  • SnapShot: Intrinsic Structural Disorder.

    abstract：:Many proteins (intrinsically disordered proteins, IDPs) or regions of proteins (intrinsically disordered regions, IDRs) lack a well-defined 3D structure under physiological conditions. Albeit unfolded and highly dynamic, these proteins are not denatured; rather, intrinsic structural disorder is their native, functiona...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2015.05.024

    authors： Guharoy M,Pauwels K,Tompa P

    更新日期：2015-05-21 00:00:00

  • ARIA, a protein that stimulates acetylcholine receptor synthesis, is a member of the neu ligand family.

    abstract：:Motor neurons stimulate their postsynaptic muscle targets to synthesize neurotransmitter receptors. Polypeptide signaling molecules may mediate this inductive interaction. Here we report the purification of ARIA, a protein that stimulates the synthesis of muscle acetylcholine receptors, and the isolation of ARIA cDNA....

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(93)90407-h

    authors： Falls DL,Rosen KM,Corfas G,Lane WS,Fischbach GD

    更新日期：1993-03-12 00:00:00

  • Inherited and De Novo Genetic Risk for Autism Impacts Shared Networks.

    abstract：:We performed a comprehensive assessment of rare inherited variation in autism spectrum disorder (ASD) by analyzing whole-genome sequences of 2,308 individuals from families with multiple affected children. We implicate 69 genes in ASD risk, including 24 passing genome-wide Bonferroni correction and 16 new ASD risk gen...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2019.07.015

    authors： Ruzzo EK,Pérez-Cano L,Jung JY,Wang LK,Kashef-Haghighi D,Hartl C,Singh C,Xu J,Hoekstra JN,Leventhal O,Leppä VM,Gandal MJ,Paskov K,Stockham N,Polioudakis D,Lowe JK,Prober DA,Geschwind DH,Wall DP

    更新日期：2019-08-08 00:00:00

  • Representation of the glomerular olfactory map in the Drosophila brain.

    abstract：:We explored how the odor map in the Drosophila antennal lobe is represented in higher olfactory centers, the mushroom body and lateral horn. Systematic single-cell tracing of projection neurons (PNs) that send dendrites to specific glomeruli in the antennal lobe revealed their stereotypical axon branching patterns and...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/s0092-8674(02)00700-6

    authors： Marin EC,Jefferis GS,Komiyama T,Zhu H,Luo L

    更新日期：2002-04-19 00:00:00

  • Crystal Structure of Antagonist Bound Human Lysophosphatidic Acid Receptor 1.

    abstract：:Lipid biology continues to emerge as an area of significant therapeutic interest, particularly as the result of an enhanced understanding of the wealth of signaling molecules with diverse physiological properties. This growth in knowledge is epitomized by lysophosphatidic acid (LPA), which functions through interactio...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2015.06.002

    authors： Chrencik JE,Roth CB,Terakado M,Kurata H,Omi R,Kihara Y,Warshaviak D,Nakade S,Asmar-Rovira G,Mileni M,Mizuno H,Griffith MT,Rodgers C,Han GW,Velasquez J,Chun J,Stevens RC,Hanson MA

    更新日期：2015-06-18 00:00:00

  • Molecular analysis of system N suggests novel physiological roles in nitrogen metabolism and synaptic transmission.

    abstract：:The amino acid glutamine has a central role in nitrogen metabolism. Although the molecular mechanisms responsible for its transport across cell membranes remain poorly understood, classical amino acid transport system N appears particularly important. Using intracellular pH measurements, we have now identified an orph...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/s0092-8674(00)81674-8

    authors： Chaudhry FA,Reimer RJ,Krizaj D,Barber D,Storm-Mathisen J,Copenhagen DR,Edwards RH

    更新日期：1999-12-23 00:00:00

  • Trans-acting nuclear protein responsible for induction of rearranged human immunoglobulin heavy chain gene.

    abstract：:A complete human gamma 1 heavy chain gene (HIG1) was transferred into mouse cells by protoplast fusion. The HIG1 gene was strongly expressed in mouse myeloma cells but not in mouse fibroblasts (L cells). Nuclear extracts from myeloma cells were injected into L cell transformants containing one copy of the HIG1 gene; t...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(86)90534-9

    authors： Maeda H,Kitamura D,Kudo A,Araki K,Watanabe T

    更新日期：1986-04-11 00:00:00

  • A Biogenesis Step Upstream of Microprocessor Controls miR-17∼92 Expression.

    abstract：:The precise control of miR-17∼92 microRNA (miRNA) is essential for normal development, and overexpression of certain miRNAs from this cluster is oncogenic. Here, we find that the relative expression of the six miRNAs processed from the primary (pri-miR-17∼92) transcript is dynamically regulated during embryonic stem c...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/j.cell.2015.07.008

    authors： Du P,Wang L,Sliz P,Gregory RI

    更新日期：2015-08-13 00:00:00

  • A nonpolymorphic class I gene in the murine major histocompatibility complex.

    abstract：:DNA sequence analysis of a class I gene (Q10), which maps to the Qa2,3 locus in the C57BL/10 (H-2b haplotype) mouse, reveals that it is almost identical to a cDNA clone (pH16) isolated from a SWR/J (H-2q haplotype) mouse liver cDNA library. Exon 5, in particular, has an unusual structure such that a polypeptide produc...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(84)90082-5

    authors： Mellor AL,Weiss EH,Kress M,Jay G,Flavell RA

    更新日期：1984-01-01 00:00:00

  • The kinky propulsion of Spiroplasma.

    abstract：:Bacteria have evolved many different means of generating movement. In this issue of Cell, Shaevitz et al. (2005) describe the swimming movement of a helical bacterium called Spiroplasma. They discover that Spiroplasma propels itself by generating two temporally distinct kinks that travel the length of the bacterium. T...

    journal_title：Cell

    pub_type： 评论,杂志文章,评审

    doi：10.1016/j.cell.2005.09.003

    authors： Wolgemuth CW,Charon NW

    更新日期：2005-09-23 00:00:00

  • Isolation of coactivators associated with the TATA-binding protein that mediate transcriptional activation.

    abstract：:A key step in the regulation of transcription involves interactions between promoter-selective factors and various components of the transcriptional apparatus. Here we report the requirements for transcriptional activation directed by NTF-1, a developmentally regulated transcription factor in Drosophila. Reconstituted...

    journal_title：Cell

    pub_type： 杂志文章

    doi：10.1016/0092-8674(81)90019-2

    authors： Dynlacht BD,Hoey T,Tjian R

    更新日期：1991-08-09 00:00:00