Abstract:
:Peroxisomes are ubiquitous eukaryotic organelles. The proteins required for peroxisome biogenesis are called peroxins, and mutations in the peroxin genes cause the devastating human developmental syndromes called the peroxisome biogenesis disorders. Our interest is in elaborating the roles that peroxisomes play in Caenorhabditis elegans development, and in establishing an invertebrate model system for the human peroxisome biogenesis disorders. The genome of C. elegans encodes homologs of 11 of the 13 human peroxins. We disrupted five nematode peroxins using RNA interference (RNAi) and found that RNAi knockdown of each one causes an early larval arrest at the L1 stage. Using a green fluorescent protein reporter targeted to the peroxisome, we establish that peroxisomal import is impaired in prx-5(RNAi) nematodes. prx-5(RNAi) animals are blocked very early in the L1 stage and do not initiate normal postembryonic cell divisions, similar to starvation-arrested larvae. Cell and axonal migrations that normally occur during the L1 stage also appear blocked. We conclude that peroxisome function is required for C. elegans postembryonic development and that disruption of peroxisome assembly by prx-5(RNAi) prevents scheduled postembryonic cell divisions. Defects in the cellular localization of peroxisomal proteins and in development are shared features of human and nematode peroxisome biogenesis disorders. In setting up a C. elegans model of peroxisomal biogenesis disorders, we suggest that genetic screens for suppression of the Prx developmental block will facilitate identification of novel intervention strategies and may provide new insights into human disease pathogenesis.
journal_name
J Cell Scijournal_title
Journal of cell scienceauthors
Thieringer H,Moellers B,Dodt G,Kunau WH,Driscoll Mdoi
10.1242/jcs.00380keywords:
subject
Has Abstractpub_date
2003-05-01 00:00:00pages
1797-804issue
Pt 9eissn
0021-9533issn
1477-9137journal_volume
116pub_type
杂志文章abstract::The small GTPase Arf4 and the Arf GTPase-activating protein (GAP) ASAP1 cooperatively sequester sensory receptor cargo into transport carriers targeted to primary cilia, but the input that drives Arf4 activation in this process remains unknown. Here, we show, by using frog retinas and recombinant human proteins, that ...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.205492
更新日期:2017-12-01 00:00:00
abstract::U937 human promonocytic leukemia cells express PKC isozymes beta 1, beta 2, epsilon and zeta. Indirect immunocytofluorescence using affinity-purified PKC-specific antibodies indicates that each of the endogenous PKC isozymes in U937 cells display a unique compartmentalization within the intact cell. PKC-beta 1 is dist...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1995-03-01 00:00:00
abstract::Previously we have shown that the Saccharomyces cerevisiae CCZ1 (YBR131w) gene encodes a protein involved in protein trafficking. Deletion of this gene leads to fragmentation of the vacuole typical of the class B vps mutants. Genetic and biochemical data indicated that Ccz1p is required for fusion of various transport...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:2001-09-01 00:00:00
abstract::Integral membrane and secretory proteins which fail to fold productively are retained in the endoplasmic reticulum and targeted for degradation by cytoplasmic proteasomes. Genetic and biochemical analyses suggest that substrates of this pathway must be dislocated across the membrane of the endoplasmic reticulum (ER) b...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:2002-07-15 00:00:00
abstract::The invasiveness of mouse lymphocytes and thymocytes, rabbit peritoneal neutrophil granulocytes (PMNs), mouse peritoneal macrophages (both activated and non-activated) and pig endothelial cells was assayed by implanting these cells to the chick wing bud. Cells of each type moved into the wing mesenchyme, although acti...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1979-12-01 00:00:00
abstract::Sexual dimorphism has been used to describe morphological differences between the sexes, but can be extended to any biologically related process that varies between males and females. The synaptonemal complex (SC) is a tripartite structure that connects homologous chromosomes in meiosis. Here, aided by super-resolutio...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.212548
更新日期:2018-03-06 00:00:00
abstract::Euglena gracilis is a suitable model system to investigate the role of zinc in the process of cell division. In zinc-deficient organisms there is a characteristic arrest of cellular proliferation, the DNA content of the cells doubles, whereas RNA and protein contents decrease. The present investigations include the gr...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1975-01-01 00:00:00
abstract::Endosomal sorting of internalized cell surface receptors to the lysosomal pathway plays a crucial role in the control of cell signaling and function. Here we report the identification of GABA(A) receptor interacting factor-1 (GRIF1), a recently discovered protein of unknown function, as a new regulator of endosome-to-...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.03249
更新日期:2006-11-15 00:00:00
abstract::S100 proteins have attracted great interest in recent years because of their cell- and tissue-specific expression and association with various human pathologies. Most S100 proteins are small acidic proteins with calcium-binding domains - the EF hands. It is thought that this group of proteins carry out their cellular ...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:2002-08-01 00:00:00
abstract::It is becoming clear that mechanical stimuli are crucial factors in regulating the biology of the cell, but the short-term structural response of a cell to mechanical forces remains relatively poorly understood. We mechanically stimulated cells transiently expressing actin-EGFP with controlled forces (0-20 nN) in orde...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.088302
更新日期:2012-02-01 00:00:00
abstract::Little is known of the molecular basis for the diversity of microtubule structure and function found within the eukaryotic flagellum. Antibodies that discriminate between tyrosinated alpha tubulin and post-translationally detyrosinated alpha tubulin were used to localize these complementary tubulin isoforms in flagell...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1998-02-01 00:00:00
abstract::Recent studies have shown that Src-family kinases (SFKs) play an important role in mediating integrin signalling, and the beta3 subunit of alphaIIbbeta3 integrin has been shown to interact with multiple SFK members. Here, we analyzed the interactions and functional consequences of Fyn and Src binding to alphaIIbbeta3....
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.014076
更新日期:2008-05-15 00:00:00
abstract::Perilipin family proteins (Plins) coat the surface of intracellular neutral lipid storage droplets in various cell types. Studies across diverse species demonstrate that Plins regulate lipid storage metabolism through recruitment of lipases and other regulatory proteins to lipid droplet surfaces. Mammalian genomes hav...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.104943
更新日期:2012-09-01 00:00:00
abstract::Gp78 (also known as AMFR and RNF45) is an E3 ubiquitin ligase that targets proteins for proteasomal degradation through endoplasmic reticulum (ER)-associated degradation (ERAD). In this study, we showed that gp78-mediated ubiquitylation is initiated in the peripheral ER. Substrate monoubiquitylation and gp78 CUE domai...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.096396
更新日期:2012-04-01 00:00:00
abstract::Several studies have shown that altered gravity conditions influence mammalian cell growth and differentiation. The molecular mechanisms underlying these effects, however, remain relatively obscure. In this paper we show that microgravity reached in a sounding rocket strongly decreases epidermal growth factor (EGF)-in...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1990-09-01 00:00:00
abstract::Human procathepsin D carries two N-linked glycosylation sites at asparagine residues 70 and 199, widely separated on the surface of the folded protein. We created monoglycosylated procathepsin D molecules by site-directed mutagenesis in vitro of the individual glycosylation sites. With only two exceptions, all 12 muta...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1995-05-01 00:00:00
abstract::To examine the relative roles of chromosomes, spindle poles and microtubules in the formation of the metaphase spindle and metakinesis, I have experimentally placed an extra centrosome-free pronucleus close to a forming bipolar spindle in a living cell. The chromosomes from the extra nucleus induce the formation of an...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1992-09-01 00:00:00
abstract::The translationally controlled protein P23 was discovered by the early induction of its rate of synthesis after mitogenic stimulation of mouse fibroblasts. P23 is expressed in almost all mammalian tissues and it is highly conserved between animals, plants and yeast. Based on its amino acid sequence, P23 cannot be attr...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1999-04-01 00:00:00
abstract::The cloned, SV40-immortalized mouse macrophage cell line, BAC1.2F5, resembles primary macrophages in its dependence on colony-stimulating factor-1 (CSF-1) for both viability and proliferation. Re-addition of CSF-1 stimulates rapid, transient behavioural changes in starved cells, which are rounded, with diffusely organ...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1989-07-01 00:00:00
abstract::The expression of the brush border-associated hydrolase sucrase-isomaltase was shown to increase from early to late passages of Caco-2 cells, concomitant with a decrease in the rates of glucose consumption. Twenty-six clones were isolated from early (P29) and late (P198) passages of the cell line. These clones show co...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1994-01-01 00:00:00
abstract::Sorting endosomes (SEs) are the regulatory hubs for sorting cargo to multiple organelles, including lysosome-related organelles, such as melanosomes in melanocytes. In parallel, melanosome biogenesis is initiated from SEs with the processing and sequential transport of melanocyte-specific proteins toward maturing mela...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.216226
更新日期:2018-09-20 00:00:00
abstract::The HB2 cell line, developed from luminal epithelial cells cultured from milk, forms ball-like structures in collagen gels which show a uniform branching response to hepatocyte growth factor. The alpha2beta1 integrin is the major integrin expressed by luminal epithelial cells, and the role of this integrin in mammary ...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1998-02-01 00:00:00
abstract::Mammalian, or mechanistic, target of rapamycin complex 2 (mTORC2) regulates a variety of vital cellular processes, and its aberrant functioning is often associated with various diseases. Rictor is a peculiar and distinguishing mTORC2 component playing a pivotal role in controlling its assembly and activity. Among exta...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.236505
更新日期:2019-11-14 00:00:00
abstract::The mechanical implementation of phagocytosis requires a well-coordinated deployment of cytoplasm and membrane during the creation of a phagosome. We follow the time course of this process in initially round passive neutrophils presented with antibody-coated beads of radii 1.1 to 5.5 microm. In particular, we monitor ...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.02275
更新日期:2005-05-01 00:00:00
abstract::Maternally inherited cytoplasmic bacteria have occasionally been observed in embryos and adults of different strains of several Drosophila species. While there is a considerable body of data on the relationship between bacteria and embryo viability, little is known about the behavior of these bacteria during the early...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1994-03-01 00:00:00
abstract::The PX domain, which until recently was an orphan domain, has emerged as the latest member of the phosphoinositide-binding module superfamily. Structural studies have revealed that it has a novel fold and identified key residues that interact with the bound phosphoinositide, enabling some prediction of phosphoinositid...
journal_title:Journal of cell science
pub_type: 杂志文章,评审
doi:
更新日期:2002-03-15 00:00:00
abstract::Recent evidence suggests that organization of the extracellular matrix (ECM) into aligned fibrils or fibril-like ECM topographies promotes rapid migration in fibroblasts. However, the mechanisms of cell migration that are altered by these changes in micro-environmental topography remain unknown. Here, using 1D fibrill...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.098806
更新日期:2012-05-01 00:00:00
abstract::Dynamins are a conserved family of proteins involved in membrane fusion and fission. Although mammalian dynamins are known to be involved in several membrane-trafficking events, the role of dynamin-1 in endocytosis is the best-characterised role of this protein family. Despite many similarities between endocytosis in ...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:10.1242/jcs.070508
更新日期:2010-10-15 00:00:00
abstract::Organelle transport is vital for the development and maintenance of axons, in which the distances between sites of organelle biogenesis, function, and recycling or degradation can be vast. Movement of mitochondria in axons can serve as a general model for how all organelles move: mitochondria are easy to identify, the...
journal_title:Journal of cell science
pub_type: 杂志文章,评审
doi:10.1242/jcs.02745
更新日期:2005-12-01 00:00:00
abstract::Melanotransferrin (p97) is an iron-binding membrane glycoprotein with 40% homology to transferrin and lactoferrin. It was first identified on the basis of its high level of expression in melanoma cells, as compared to normal melanocytes. It is also present in many cultured cell types. In normal tissues, p97 is express...
journal_title:Journal of cell science
pub_type: 杂志文章
doi:
更新日期:1993-04-01 00:00:00