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 Mito-mice, the Delta mtDNA4696 is transmitted maternally, and induces mitochondrial dysfunction in various tissues. Most of the Mito-mice with high proportions of the Delta mtDNA4696 died at about age 6 months due to renal failure. Mito-mice are the first animal model for mtDNA-based diseases and will be valuable for studying pathogenesis and for identifying effective drug and gene therapies.
journal_name
Semin Cell Dev Bioljournal_title
Seminars in cell & developmental biologyauthors
Nakada K,Inoue K,Hayashi JIdoi
10.1006/scdb.2001.0283subject
Has Abstractpub_date
2001-12-01 00:00:00pages
459-65issue
6eissn
1084-9521issn
1096-3634pii
S1084-9521(01)90283-6journal_volume
12pub_type
杂志文章abstract：:The extracellular matrix (ECM) consists of a complex mixture of structural and functional proteins and serves an important role in tissue and organ morphogenesis, maintenance of cell and tissue structure and function, and in the host response to injury. Xenogeneic and allogeneic ECM has been used as a bioscaffold for ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/s1084952102000940
更新日期：2002-10-01 00:00:00
abstract：:Autophagy, a conserved self-eating process for the bulk degradation of cytoplasmic materials, involves double-membrane autophagosomes formed when an isolation membrane emerges and their direct fusion with lysosomes for degradation. For the early biogenesis of autophagosomes and their later degradation in lysosomes, me...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2016.07.009
更新日期：2016-12-01 00:00:00
abstract：:In all organisms tRNAs play the essential role of connecting the genetic information found in DNA with the protein synthesis machinery ensuring fidelity during translation. Following transcription tRNAs undergo a number of processing events including numerous post-transcriptional modifications that render a tRNA molec...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2011.10.009
更新日期：2012-05-01 00:00:00
abstract：:During eye formation, inductive phenomena occurring between retinal pigmented epithelium (RPE) and retina are not well understood. After briefly summarizing the normal development of retina and RPE, we present three-dimensional in vitro models of the chick embryonic retina which allows elucidation of RPE-retina intera...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1006/scdb.1998.0234
更新日期：1998-06-01 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：:Phytochromes in harmony with blue light photoreceptors play a major role in controlling plant growth and development from germination to seed maturation. Light absorption by phytochromes triggers a signaling cascade, phototransduction, which culminates in regulated gene expression. A major regulatory step at the cellu...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1006/scdb.2000.0202
更新日期：2000-12-01 00:00:00
abstract：:α-synuclein and Tau are proteins prone to pathological misfolding and aggregation that are normally found in the presynaptic and axonal compartments of neurons. Misfolding initiates a homo-oligomerization and aggregation cascade culminating in cerebral accumulation of aggregated α-synuclein and Tau in insoluble protei...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.05.005
更新日期：2020-03-01 00:00:00
abstract：:Canalization, or phenotypic robustness in the face of environmental and genetic perturbation, is an emergent property of living systems. Although this phenomenon has long been recognized, its molecular underpinnings have remained enigmatic until recently. Here, we review the contributions of the molecular chaperone Hs...
journal_title：Seminars in cell & developmental biology
pub_type： 历史文章,杂志文章,评审
doi：10.1016/j.semcdb.2018.05.015
更新日期：2019-04-01 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：:Lipids are now widely considered to play a variety of important roles in T-cell mediated immunity, including serving as antigens. Lipid-based antigens are presented by a specialised group of glycoproteins termed CD1. In humans, three classes of CD1 molecules exist: group 1 (CD1a, CD1b, CD1c), group 2 (CD1d), and group...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2017.11.002
更新日期：2018-12-01 00:00:00
abstract：:Correct orientation of the mitotic spindle is crucial for the proper segregation of localized determinants and the correct spatial organization of cells in early embryos. The cues dividing cells use to orient their mitotic spindles are currently the subject of intensive investigation in a number of model systems. One ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2006.04.005
更新日期：2006-04-01 00:00:00
abstract：:Lipid droplets (LDs) are versatile organelles with central roles in lipid and energy metabolism in all eukaryotes. They primarily buffer excess fatty acids by storing them as neutral lipids, mainly triglycerides and steryl esters. The neutral lipids form a core, surrounded by a unique phospholipid monolayer coated wit...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2020.03.001
更新日期：2020-12-01 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 urinary collecting system is derived from an epithelial protrusion arising from the Wolffian duct called the ureteric bud (UB) by the signal from its inductive tissue, metanephric mesenchyme (MM). Targeted gene mutation studies have shown that several transcription factors and MM-secreted glial cell line-derived n...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/s1084-9521(03)00024-7
更新日期：2003-08-01 00:00:00
abstract：:The vertebrate ocular lens is a fascinating and unique transparent tissue that grows continuously throughout life. During the process of differentiation into fiber cells, lens epithelial cells undergo dramatic morphological changes, membrane remodeling, polarization, transcriptional activation and elimination of cellu...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2006.10.011
更新日期：2006-12-01 00:00:00
abstract：:Mammalian spermatogenesis is a tightly coordinated process that gives rise to mature spermatozoa capable of fertilising an ovum during sexual reproduction. A population of stem and progenitor cells known as undifferentiated spermatogonia enables continual spermatogenesis throughout life. A complex transcriptional netw...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2017.10.006
更新日期：2018-07-01 00:00:00
abstract：:The Notch signaling pathway is an evolutionarily conserved pathway that is critical for tissue morphogenesis during development, but is also involved in tissue maintenance and repair in the adult. In skeletal muscle, regulation of Notch signaling is involved in somitogenesis, muscle development, and the proliferation ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2005.07.002
更新日期：2005-08-01 00:00:00
abstract：:Mitochondria are the key energy-producing organelles and cellular source of reactive species. They are responsible for managing cell life and death by a balanced homeostasis passing through a network of structures, regulated principally via fission and fusion. Herein we discuss about the most advanced findings conside...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2019.05.022
更新日期：2020-02-01 00:00:00
abstract：:Yeast prions are atypical genetic elements that are transmitted as heritable protein conformations. [PSI+], [URE3], and [PIN+] are three well-studied prions in the budding yeast, Saccharomyces cerevisiae. In the last three years, several additional prions have been reported in yeast, including [SWI+], [OCT+], [MCA], [...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2011.03.003
更新日期：2011-07-01 00:00:00
abstract：:The extracellular matrix (ECM) of blood vessels, which is composed of both the vascular basement membrane (BM) and the interstitial ECM is identified as a crucial component of the vasculature. We here focus on the unique molecular composition and scaffolding of the capillary ECM, which provides structural support to b...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.08.007
更新日期：2019-05-01 00:00:00
abstract：:Recent appraisals of mesenteric anatomy clarify its structure and show a continuous and helical-shaped organ. This new model signifies a departure from the conventional model which described multiple, separate "mesenteries". Renaissance anatomists depicted the mesentery as a continuous structure. Events that led to re...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2018.10.003
更新日期：2019-08-01 00:00:00
abstract：:The members of the large family of claudin proteins regulate ion and water flux across the tight junction. Many claudins, e.g. claudins 2 and 15, accomplish this by forming size- and charge-selective paracellular channels. Claudins also appear to be essential for genesis of tight junction strands and recruitment of ot...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2015.04.008
更新日期：2015-06-01 00:00:00
abstract：:Perinatal environment plays a crucial role in brain development and determines its function through life. Epidemiological studies and clinical reports link perinatal exposure to infection and/or immune activation to various psychiatric disorders. In addition, accumulating evidence from animal models shows that perinat...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2017.09.010
更新日期：2018-05-01 00:00:00
abstract：:Axonal projections between two populations of neurons, which preserve neighborhood relationships, are called topographic. They are ubiquitous in the brain. The development of the retinotectal projection, mapping the retinal output onto the roof of the midbrain, has been studied for decades as a model system. The rigid...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2014.07.010
更新日期：2014-11-01 00:00:00
abstract：:Low oxygen adaptation is essential for aerobic fungi that must survive in varied oxygen environments. Pathogenic fungi in particular must adapt to the low oxygen host tissue environment in order to cause infection. Maintenance of lipid homeostasis is especially important for cell growth and proliferation, and is a hig...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2017.08.043
更新日期：2018-09-01 00:00:00
abstract：:Paramutation was initially described in maize and was defined as an epigenetic interaction between two alleles of a locus, through which one allele induces a heritable modification of the other allele without modifying the DNA sequence [1,2]. Thus it implies that the paramutated allele conserves its new properties on ...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2015.08.009
更新日期：2015-08-01 00:00:00
abstract：:Regulation of Notch signaling is critical to development and maintenance of most eukaryotic organisms. The Notch receptors and ligands are integral membrane proteins and direct cell-cell interactions are needed to activate signaling. Ligand-expressing cells activate Notch signaling through an unusual mechanism involvi...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2012.01.011
更新日期：2012-06-01 00:00:00
abstract：:In order for a successful pregnancy to occur, the embryo must attach to the luminal epithelial cells and invade into the stroma. Then, the surrounding stromal cells need to undergo decidualization in order to establish the vasculature necessary for survival of the embryo. These events in early pregnancy are tightly re...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2007.12.001
更新日期：2008-04-01 00:00:00
abstract：:Polycystic kidney disease (PKD), comprising autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD), is characterized by incessant cyst formation in the kidney and liver. ADPKD and ARPKD represent the leading genetic causes of renal disease in adults and children,...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2020.05.003
更新日期：2020-05-28 00:00:00
abstract：:Influenza viral particles are enveloped by a lipid bilayer. A major step in infection is fusion of the viral and host cellular membranes, a process with large kinetic barriers. Influenza membrane fusion is catalyzed by hemagglutinin (HA), a class I viral fusion protein activated by low pH. The exact nature of the HA c...
journal_title：Seminars in cell & developmental biology
pub_type： 杂志文章,评审
doi：10.1016/j.semcdb.2016.07.007
更新日期：2016-12-01 00:00:00
