Abstract：
:The number of human genetic disorders shown to be due to defects in membrane trafficking has greatly increased during the past five years. Defects have been identified in components involved in sorting of cargo into transport carriers, vesicle budding and scission, movement of vesicles along cytoskeletal tracks, as well as in vesicle tethering, docking and fusion at the target membrane. The nervous system is extremely sensitive to such disturbances of the membrane trafficking machinery, and the majority of these disorders display neurological defects--particularly diseases affecting the motility of transport carriers along cytoskeletal tracks. In several disorders, defects in a component that represents a fundamental part of the trafficking machinery fail to cause global transport defects but result in symptoms limited to specific cell types and transport events; this apparently reflects the redundancy of the transport apparatus. In groups of closely related diseases such as Hermansky-Pudlak and Griscelli syndromes, identification of the underlying gene defects has revealed groups of genes in which mutations lead to similar phenotypic consequences. New functionally linked trafficking components and regulatory mechanisms have thus been discovered. Studies of the gene defects in trafficking disorders therefore not only open avenues for new therapeutic approaches but also significantly contribute to our knowledge of the fundamental mechanisms of intracellular membrane transport.
journal_name
J Cell Scijournal_title
Journal of cell scienceauthors
Olkkonen VM,Ikonen Edoi
10.1242/jcs.03303subject
Has Abstractpub_date
2006-12-15 00:00:00pages
5031-45issue
Pt 24eissn
0021-9533issn
1477-9137pii
119/24/5031journal_volume
119pub_type
杂志文章,评审abstract：:Annexin A2 (AnxA2) is a multi-functional and -compartmental protein whose subcellular localisation and functions are tightly regulated by its post-translational modifications. AnxA2 and its Tyr23-phosphorylated form (pTyr23AnxA2) are involved in malignant cell transformation, metastasis and angiogenesis. Here, we show...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.173195
更新日期：2016-01-15 00:00:00
abstract：:We have cloned and characterized a single copy C. reinhardtii gene containing an open reading frame of 333 nucleotides encoding a 12.7 kDa protein. The novel protein, DIP13, exhibits 60% identity with two mammalian proteins, human NA14 and an unnamed mouse protein. Homologous sequences are also present in several prot...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.00337
更新日期：2003-04-15 00:00:00
abstract：:Epithelial cell-cell junctions are specialised structures connecting individual cells in epithelial tissues. They are dynamically and functionally linked to the actin cytoskeleton. Disassembly of these junctions is a key event during physiological and pathological processes, but how this influences gene expression is ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.014456
更新日期：2008-04-01 00:00:00
abstract：:The complex formed by Rad9, Rad1 and Hus1 (9-1-1) protects against genomic instability by activating DNA damage checkpoint and DNA damage repair pathways, mainly in response to replication fork collapse and UV lesions. Here we compare the role of Rad9A (also known as Rad9) with the human paralogue Rad9B. Unlike Rad9A,...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.091124
更新日期：2012-03-01 00:00:00
abstract：:Cells that are treated long-term with TNFalpha or short-term with TGFalpha together with cycloheximide (CHX) undergo apoptosis. Cell shrinkage and detachment during apoptosis is dependent on actomyosin contractility. Myosin II heavy chain (MHCII) isoforms have shared and distinct functions. Here, we investigated wheth...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.022640
更新日期：2008-05-15 00:00:00
abstract：:Trypsin digestion of demembranated fowl spermatozoa caused a longitudinal splitting of the distal part of the axoneme. The resulting strands, consisting of groups of doublet microtubules, formed left-handed helices. On the evidence of electron micrographs, the digestion had caused the loss of dynein arms from the oute...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1987-10-01 00:00:00
abstract：:The binding of GFP-tagged tetracycline repressor (TetR) molecules to chromosomally integrated tetracycline operator (tetO) sequence repeats has been used as a system to study chromosome behaviour microscopically in vivo. We found that these integrated transgenes influence the architecture of yeast interphase nuclei, a...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：2002-03-15 00:00:00
abstract：:To optimally perform the diversity of metabolic functions that occur within peroxisomes, cells must dynamically regulate peroxisome size, number and content in response to the cell state and the environment. Except for transcriptional regulation little is known about the mechanisms used to perform this complicated fea...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.195255
更新日期：2016-11-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章,评审
doi：10.1242/jcs.146258
更新日期：2015-01-15 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1994-03-01 00:00:00
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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
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1986-03-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.02684
更新日期：2005-12-15 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1993-06-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1977-06-01 00:00:00
abstract：:The marginal band microtubules of isolated Xenopus erythrocyte cytoskeletons possess the stability properties of non-steady-state microtubules. They are unperturbed by low temperatures, a variety of microtubule inhibitors, hypotonic treatment and the direct action of calcium. These microtubules can be rapidly depolyme...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1985-11-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.044305
更新日期：2009-11-15 00:00:00
abstract：:By differential screening of a Xenopus laevis egg cDNA library, we have isolated a 2,111 bp cDNA which corresponds to a maternal mRNA specifically deadenylated after fertilisation. This cDNA, called Eg2, encodes a 407 amino acid protein kinase. The pEg2 sequence shows significant identity with members of a new protein...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1998-03-01 00:00:00
abstract：:Mammalian Staufen2 (Stau2) is involved in mRNA transport in neurons. Here, we report that Stau2 is a double-stranded RNA-binding protein that is mainly expressed in the brain. We show that Stau2 is found in the somatodendritic compartment of neurons. In dendrites, Stau2 is aligned on individual tracts and colocalizes ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：2002-08-15 00:00:00
abstract：:Extracellular matrix receptors of the integrin family have a dual role in the epidermis, regulating both adhesion and differentiation. Loss of contact with the extracellular matrix causes keratinocytes to become committed to terminal differentiation, and results in a decrease in the ability of the alpha 5 beta 1 integ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1993-12-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.00948
更新日期：2004-03-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章,评审
doi：
更新日期：2002-05-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.096941
更新日期：2012-07-15 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：2000-11-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章,评审
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更新日期：2004-05-01 00:00:00
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journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1993-04-01 00:00:00
abstract：:Using a reconstituted complex of profilin and skeletal muscle actin as an antigen, we generated a monoclonal mouse antibody against actin, termed 2G2. As revealed by immunoblots of proteolytic actin fragments and by pepscan analysis, the antibody recognises a nonsequential epitope on actin which is located within thre...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1999-03-01 00:00:00
abstract：:Adaptive immunity, a vertebrate specialization, adds memory and exquisite specificity to the basic innate immune responses present in invertebrates while conserving metabolic resources. In adaptive immunity, antigenic challenge requires extremely rapid proliferation of rare antigen-specific lymphocytes to produce larg...
journal_title：Journal of cell science
pub_type： 杂志文章,评审
doi：10.1242/jcs.096040
更新日期：2012-03-15 00:00:00
abstract：:Structures retaining many of the morphological features of nuclei may be released by gently lysing human cells in solutions containing non-ionic detergents and high concentrations of salt. These nucleoids contain superhelical DNA. Using a double-labelling procedure we have compared, at different salt concentrations, t...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1978-02-01 00:00:00
abstract：:Nerve branching is controlled by intrinsic and extrinsic cues, one of which may be a small applied electric field. Lateral processes were induced by passing current through a micropipette placed at 90 degrees to the shaft of a developing nerve. The appearance of processes was a polarised event with a large majority ar...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1990-04-01 00:00:00
