Abstract：
:The endoplasmic reticulum (ER) is an intracellular compartment devoted to the synthesis, segregation and folding of soluble and membrane secretory proteins. Some mutations in these proteins lead to their incorrect or incomplete folding in the ER. The ER has a quality control system which detects misfolded proteins and then specifies their fate. Some mutated proteins are retained in the ER wherein they accumulate (Russell bodies for misfolded immunoglobulin heavy chains, the PiZZ for alpha 1-antitrypsin), others are retrotranslocated from the ER and degraded by the cytosolic proteasomal system, and yet other proteins are eventually secreted (in AZC-treated cells). In this review we summarize the role of ER resident proteins in quality control of mutated secretory proteins.
journal_name
Semin Cell Dev Bioljournal_title
Seminars in cell & developmental biologyauthors
Chevet E,Jakob CA,Thomas DY,Bergeron JJdoi
10.1006/scdb.1999.0316subject
Has Abstractpub_date
1999-10-01 00:00:00pages
473-80issue
5eissn
1084-9521issn
1096-3634pii
S1084-9521(99)90316-6journal_volume
10pub_type
杂志文章,评审abstract：:During development cranial neural crest cells (NCCs) display a striking transition from collective to single-cell migration, but the mechanisms enabling individual NCCs to separate from the neural crest tissue are still incompletely understood. In this study we have employed atomic force microscopy (AFM) to investigat...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2017.08.058
更新日期：2018-01-01 00:00:00
abstract：:Embryonic patterning of the mouse during gastrulation and early organogenesis engenders the specification of anterior versus posterior structures and body laterality by the interaction of signalling and modulating activities. A group of cells in the mouse gastrula, characterised by the expression of a repertoire of "o...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2004.04.005
更新日期：2004-10-01 00:00:00
abstract：:The endocytic pathway is essential for processes that define how cells interact with their environment, including receptor signalling, cell adhesion and migration, pathogen entry, membrane protein turnover and nutrient uptake. The spatial organisation of endocytic trafficking requires motor proteins that tether membra...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2014.04.011
更新日期：2014-07-01 00:00:00
abstract：:Indiscriminate use of synthetic chemical compounds and the unregulated presence of heavy metals threatens the integral reproducibility of mankind and other living organisms. The toxicity of these compounds far outweighs the usefulness of these compounds. Male reproductive health is linked to the process of spermatogen...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2016.03.024
更新日期：2016-11-01 00:00:00
abstract：:Freshwater planarians are well known for their remarkable plasticity and regenerative capabilities. Most studies of planarian regeneration have specifically examined regeneration after transverse or longitudinal sectioning or during homeostasis in intact adults. However, tissue transplantation, first performed over a ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.05.022
更新日期：2019-03-01 00:00:00
abstract：:Large-scale identification of elements associated with gene expression revealed that many of them are located extremely far from gene transcriptional start sites. We review here the growing evidence that show that distal cis-acting elements provide key instructions to genes, as genetic variation affecting them is grow...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
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更新日期：2016-09-01 00:00:00
abstract：:Eph receptor tyrosine kinases mediate cell-cell communication by interacting with ephrin ligands residing on adjacent cell surfaces. In doing so, these juxtamembrane signaling complexes provide important contextual information about the cellular microenvironment that helps orchestrate tissue morphogenesis and maintain...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2011.10.017
更新日期：2012-02-01 00:00:00
abstract：:The family Cichlidae contains approximately 2000 species that live in diverse freshwater habitats including murky lakes, turbid rivers, and clear lakes from both the Old and New Worlds. Their visual systems are similarly diverse and have evolved specific sensitivities that differ along several axes of variation. Varia...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2020.04.015
更新日期：2020-10-01 00:00:00
abstract：:Most mammals cannot easily overcome degenerative disease or traumatic injuries. In contrast, an innate ability to regenerate is observed across animal phyla. Freshwater planarians are amongst the organisms that are capable of stem cell-mediated whole-body regeneration and have served as an exemplary model to study how...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.04.009
更新日期：2019-03-01 00:00:00
abstract：:Acquisition of the pluripotent state coincides with epigenetic reprogramming of the X-chromosome. Female embryonic stem cells are characterized by the presence of two active X-chromosomes, cell differentiation by inactivation of one of the two Xs, and induced pluripotent stem cells by reactivation of the inactivated X...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2011.02.025
更新日期：2011-06-01 00:00:00
abstract：:Embryonic mammary gland development involves the formation of mammary placodes, invagination of flask-shaped mammary buds and development of miniature bi-layered ductal trees. Currently there is a good understanding of the factors that contribute to ectodermal cell movements to create these appendages and of pathways ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2020.12.012
更新日期：2021-01-17 00:00:00
abstract：:Control of translation allows for the production of stoichiometric levels of each protein in the cell. Attaining such a level of fine-tuned regulation of protein production requires the coordinated temporal and spatial control of numerous cellular signalling cascades impinging on the various components of the translat...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2014.09.014
更新日期：2014-12-01 00:00:00
abstract：:Pigment cells serve a variety of important uses across the animal kingdom, and in many species can change and regenerate throughout the lifetime of the organism. The functions of these cells, as well as their origins in both embryonic development and adult regeneration, are not fully understood. Here, we review advanc...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.05.010
更新日期：2019-03-01 00:00:00
abstract：:The vast amount of recent progress made on the sequence of the human genome has allowed an unprecedented examination of cis-regulatory networks. These networks consist of functional elements such as promoters, enhancers, silencers, and insulators, and their coordinated activity is responsible for regulation of gene ex...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2009.06.005
更新日期：2009-09-01 00:00:00
abstract：:The TGF-β ligand superfamily contains at least 40 members, many of which are produced and act within the mammalian testis to facilitate formation of sperm. Their progressive expression at key stages and in specific cell types determines the fertility of adult males, influencing testis development and controlling germl...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2015.10.029
更新日期：2015-09-01 00:00:00
abstract：:Fibrillin microfibrils are extensible polymers that endow connective tissues with long-range elasticity and have widespread distributions in both elastic and non-elastic tissues. They act as a template for elastin deposition during elastic fibre formation and are essential for maintaining the integrity of tissues such...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.07.016
更新日期：2019-05-01 00:00:00
abstract：:Caspases are cysteine proteases that play important and well-defined roles in apoptosis and inflammation. Increasing evidence point to alternative functions of caspases where restricted and localized caspase activation within neurons allows for a variety of non-apoptotic and non-inflammatory processes required for bra...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2017.11.037
更新日期：2018-10-01 00:00:00
abstract：:Understanding how some animals are immortal and avoid the ageing process is important. We currently know very little about how they achieve this. Research with genetic model systems has revealed the existence of conserved genetic pathways and molecular processes that affect longevity. Most of these established model o...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2017.08.028
更新日期：2017-10-01 00:00:00
abstract：:We have successfully produced "Mito-mice" harbouring a pathogenic mtDNA mutation. We generated the mice by introducing mitochondria with a 4696 base-pair mtDNA deletion (Delta mtDNA4696) into mouse embryos. This deletion encompasses nucleotides 7759-12 454 and includes six tRNA genes and seven structural genes. In Mit...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章
doi：10.1006/scdb.2001.0283
更新日期：2001-12-01 00:00:00
abstract：:Studies of TRH and GnRH receptors have revealed much information about the roles of G-proteins and beta-arrestins, as well as receptor residues important for signaling, desensitization and internalization. However, the proteins involved are only just beginning to be identified and characterized. Additional complexity ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2003.12.016
更新日期：2004-06-01 00:00:00
abstract：:Neural stem cells (NSCs) are subscribed extraordinary potential in repair of the damaged nervous system. However, the molecular identity of NSCs has not been established. Most NSC cultures contain large numbers of multipotent, bipotent, and lineage restricted neural progenitors, the majority of which appear to lose ne...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/s1084-9521(03)00007-7
更新日期：2003-06-01 00:00:00
abstract：:The functional unit of the mammalian metanephric kidney is the nephron: a complex tubular structure dedicated to blood filtration and maintenance of several important physiological functions. Nephrons are assembled from a nephron-restricted pool of mesenchymal progenitors over an extensive developmental period that is...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2014.08.014
更新日期：2014-12-01 00:00:00
abstract：:In the crowded intranuclear environment, entropic depletion forces between macromolecules are expected to be strong. A review of simulations of linear polymers leads to several predictions about probable conformations of a polynucleosome chain in these conditions. These include a globular conformation, variable compac...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2007.08.006
更新日期：2007-10-01 00:00:00
abstract：:Glutathione (GSH) is the most abundant antioxidant and a major detoxification agent in cells. It is synthesized through two-enzyme reaction catalyzed by glutamate cysteine ligase and glutathione synthetase, and its level is well regulated in response to redox change. Accumulating evidence suggests that GSH may play im...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2012.03.017
更新日期：2012-09-01 00:00:00
abstract：:Gap junctions and hemichannels comprised of connexins influence epidermal proliferation and differentiation. Significant advances in our understanding of the functional role of connexins in the skin have been made by studying the diseases caused by connexin mutations. Eleven clinically defined cutaneous disorders with...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2015.11.018
更新日期：2016-02-01 00:00:00
abstract：:The pancreas is a compound gland comprised of both exocrine acinar and duct cells as well as endocrine islet cells. Most notable amongst the latter are the insulin-synthesizing β-cells, loss or dysfunction of which manifests in diabetes mellitus. All exocrine and endocrine cells derive from multipotent pancreatic prog...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.08.008
更新日期：2019-08-01 00:00:00
abstract：:Gene duplication events produce both perfect and imperfect copies of genes. Perfect copies are said to be functionally redundant when knockout of one gene produces no 'scoreable', phenotypic effects. Preserving identical, duplicate copies of genes is problematic as all copies are prone to accumulate neutral mutations ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1006/scdb.1999.0337
更新日期：1999-10-01 00:00:00
abstract：:No abstractCopyright 1997 Academic Press Limited Copyright 1997Academic Press Limited ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章
doi：10.1006/scdb.1997.0170
更新日期：1997-10-01 00:00:00
abstract：:Cardiac chamber walls contain large numbers of non-contractile interstitial cells, including fibroblasts, endothelial cells, pericytes and significant populations of blood lineage-derived cells. Blood cells first colonize heart tissues a few days before birth, although their recruitment from the bloodstream to the car...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2020.06.008
更新日期：2020-06-23 00:00:00
abstract：:The first lineage segregation event in mouse embryos produces two separate cell populations: inner cell mass and trophectoderm. This is understood to be brought about by cells sensing their position within the embryo and differentiating accordingly. The cellular and molecular underpinnings of this process remain under...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2015.09.006
更新日期：2015-12-01 00:00:00
