Abstract：
:Perforin, a pore-forming protein toxin synthesized and stored in the cytoplasmic vesicles of cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells, is secreted when these effector lymphocytes encounter virus-infected or neoplastic cells. Perforin is encoded by a single-copy gene and is critical for immune homeostasis and defense of the organism against intracellular sepsis. A complete deficiency of perforin expression in either mice or humans is associated with a syndrome of immune insufficiency and severely deregulated lymphoid homeostasis. Humans who inherit inactivating mutations of perforin or defects in various parts of the cellular machinery that delivers perforin to the target cell suffer from familial hemophagocytic lymphohistiocytosis (FHL), a fatal condition necessitating bone marrow transplantation, usually in infancy. In mice, a high incidence of spontaneous B cell lymphoma has also been noted as the animals age. Across human populations, a number of polymorphisms that result in measurable, but suboptimal CTL activity have been noted, and some of these predispose to attenuated FHL or susceptibility to infectious disease, but in many cases, to no discernible disease predisposition. This chapter discusses the significance of human perforin polymorphisms, particularly those associated with diseases other than FHL, and recent advances in our understanding of perforin biology and function.
journal_name
Adv Exp Med Bioljournal_title
Advances in experimental medicine and biologyauthors
Trapani JA,Voskoboinik Idoi
10.1007/978-0-387-72005-0_24subject
Has Abstractpub_date
2007-01-01 00:00:00pages
235-42eissn
0065-2598issn
2214-8019journal_volume
601pub_type
杂志文章,评审abstract：:TRPV5 and TRPV6 are unique members of the TRP super family. They are highly selective for Ca(2+) ions with multiple layers of Ca(2+)-dependent inactivation mechanisms, expressed at the apical membrane of Ca(2+) transporting epithelia, and robustly responsive to 1,25-dihydroxivitamin D(3). These features are well suite...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-94-007-0265-3_14
更新日期：2011-01-01 00:00:00
abstract：:12-lipoxygenase (12-LOX) expression and function in the regulation of the metastatic phenotype was demonstrated in several murine melanoma lines before. Here we have provided novel evidences that, though at a low level (in max. 15% of the cell population), human melanoma lines (HT168, M1, HT199, HT18 and WM35) express...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4615-4793-8_89
更新日期：1999-01-01 00:00:00
abstract：:We investigated the antioxidant activity of taurine rich water extract from the cephalothorax of Fenneropenaeus chinensis (FCC). The antioxidant potency of water extract from FCC was assessed using various assay methods, such as DPPH (1,1-diphenyl-2-picrylhydrazyl), alkyl radical scavenging activity, ABTS (2,2'-azinob...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-94-024-1079-2_92
更新日期：2017-01-01 00:00:00
abstract：:Melanoma and nonmelanoma skin cancer (NMSC) are now the most common types of cancer in white populations. Both tumor entities show an increasing incidence rate worldwide but a stable or decreasing mortality rate. The rising incidence rates of NMSC are probably caused by a combination of increased sun exposure or expos...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-0-387-77574-6_8
更新日期：2008-01-01 00:00:00
abstract：:Eleven weeks after surgery, a fine microvessel network was seen in the feet of patients with arteriosclerosis obliterans treated by distal vein arterialization. A possible mechanism for establishment of blood flow to, and biological maintenance of, the graft in the foot, in relation to oxygen consumption rate during w...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4614-4989-8_34
更新日期：2013-01-01 00:00:00
abstract：:In this chapter, we intend to review gene therapy concepts applied to the potential treatment of tyrosinemia for parents and pediatricians. Therefore, our main objective is to give general informations in a comprehensible manner. Considering the nature of tyrosinemia and the current state of technology, a particular f...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-55780-9_21
更新日期：2017-01-01 00:00:00
abstract：:From the time of the Hong Kong pandemic of 1968-1969, vaccines against influenza are trivalent, containing two subtypes of influenza type A: A/H1N1/ and A/H3N2/, and influenza type B. In 1980, circulation of the new Yamagata and Victoria lineages of influenza B virus was noted. Since both lineages have continued to ci...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/5584_2015_200
更新日期：2016-01-01 00:00:00
abstract：:The function and characteristics of mouse folylpolyglutamate synthetase have been examined. Folate polyglutamates were poor substrates for the efflux mechanism for monoglutamates in L1210 mouse leukemia cells, with negligible loss of preformed folate polyglutamates to the medium over four hours. Disruption of folate m...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4757-5241-0_24
更新日期：1983-01-01 00:00:00
abstract：:The structural similarity of cholesterol oxidation products (COP) to native cholesterol and their xenobiotic effects prompt researchers to study the long-term effects of the assimilation of these compounds into our tissues. COP are present in our food system. The level of exposure changes as our food products and our ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：
更新日期：1999-01-01 00:00:00
abstract：:The expression of thymidine kinase--an enzyme of the DNA precursor pathway--is strictly regulated during the normal cellular cycle, but is much higher and permanently expressed in malignant growing cells. Using a new cytofluorometric assay for thymidine kinase in single cells, we were able to discriminate between norm...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：
更新日期：1998-01-01 00:00:00
abstract：:Supplementation of diets with free amino acids has not routinely been beneficial and poses a potential risk. Thus, mixing proteins to increase the quality of the dietary protein is attractive. The slope-ratio technique for evaluation of protein quality has serious drawbacks, because the growth response is not linear a...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4684-3366-1_20
更新日期：1978-01-01 00:00:00
abstract：:Metastasis is the major cause of breast cancer death worldwide. In metastatic breast cancer, circulating tumor cells (CTCs) can be captured from patient blood samples sequentially over time and thereby serve as surrogates to assess the biology of surviving cancer cells that may still persist in solitary or multiple me...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-030-35805-1_5
更新日期：2020-01-01 00:00:00
abstract：:Transcranial direct current stimulation (tDCS) is a noninvasive brain stimulation technique that is applied during stroke rehabilitation. The purpose of this study was to examine diachronic intracranial hemodynamic changes using near-infrared spectroscopy (NIRS) during tDCS applied to the primary motor cortex (M1). Se...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4939-3023-4_42
更新日期：2016-01-01 00:00:00
abstract：:Environmental and iatrogenic exposures contribute significantly to human diseases, including cancer. The list of known human carcinogens has recently been extended by the addition of aristolochic acids (AAs). AAs occur primarily in Aristolochia herbs, which are used extensively in folk medicines, including Traditional...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-030-41283-8_9
更新日期：2020-01-01 00:00:00
abstract：:The interpretation of cerebral tissue oxygen saturation values (StO2) in clinical settings is currently complicated by the use of different near-infrared spectrophotometry (NIRS) devices producing different StO2 values for the same oxygenation due to differences in the algorithms and technical aspects. The aim was to ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4939-3023-4_52
更新日期：2016-01-01 00:00:00
abstract：:Cinematographic recordings of sarcomere shortening were performed on glycerinated horseshoe crab muscle myofibrils during Ca-activated contraction. When the preparations at slack length (sarcomere length, 7-9 microns) were locally activated with iontophoretically applied Ca ions, the A-band length did not change appre...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4684-4703-3_10
更新日期：1984-01-01 00:00:00
abstract：:To develop an osteochondral tissue regeneration strategy it is extremely important to take into account the multiscale organization of the natural extracellular matrix. The structure and gradients of organic and inorganic components present in the cartilage and bone tissues must be considered together. Another critica...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-76711-6_6
更新日期：2018-01-01 00:00:00
abstract：:Antibiotic resistance is a global epidemic, becoming increasingly pressing due to its rapid spread. There is thus a critical need to develop new therapeutic approaches. In addition to searching for new antibiotics, looking into existing mechanisms of natural host defense may enable researchers to improve existing defe...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-030-46886-6_7
更新日期：2020-01-01 00:00:00
abstract：:Oral drug administration is the most favorable route of drug administration in the clinic. Intestinal transporters have been shown to play a significant role in the rate and extent of drug absorption of some, but not all, drug molecules. Due to the heterogeneous expression of multiple transporters along the intestine,...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-981-13-7647-4_8
更新日期：2019-01-01 00:00:00
abstract：:The medium from cultured optic tectum of the goldfish intact or at various days after optic nerve crush, or the co-culture of this optic tectum with post-crush goldfish retinal explants plating in the absence of fetal calf serum, but in the presence of glucose, modulate its outgrowth. The a dition of taurine did not f...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-0-387-33504-9_49
更新日期：2006-01-01 00:00:00
abstract：:Cancer development originates in a single mutant cell transformed from a normal cell, including further evolution of pro-tumor cells through additional mutations into malignant cancer tissues. Data from recent studies, however, suggest that most pro-tumor cells do not develop into tumors but remain dormant within or a...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-030-23629-8_5
更新日期：2019-01-01 00:00:00
abstract：:Serologic and structural comparisons of the rabbit IgA-g allotypes revealed that 1) the IgA-g allotypes have multiple allotypic determinant sites, 2) the g74, g76 and g77 allotypic specificities have several allotypic determinants in common whereas g75 molecules do not appear to have allotypic determinants in common w...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4684-3369-2_58
更新日期：1978-01-01 00:00:00
abstract：:The major central part of elastic ligaments is constituted by longitudinally orientated elastic fibres, each surrounded by a sheath of spirally orientated collagen fibrils with an intervening thin layer of microfibrils. In the terminal region of such ligaments the orientation of the collagen fibrils is longitudinal an...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4684-9093-0_9
更新日期：1977-01-01 00:00:00
abstract：:An appropriate animal model is essential to screening drugs or designing a treatment strategy for geographic atrophy. Since oxidative stress contributes to the pathological changes of the retinal pigment epithelium (RPE), we are reporting a new mouse AMD model of retinal degeneration by inducing mitochondrial oxidativ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-3-319-17121-0_5
更新日期：2016-01-01 00:00:00
abstract：:Antimicrobial resistance (AMR) is currently one of the main concerns for human health.Due to its rapid increase and global diffusion, several common microbial infections might become not curable in the future decades, making it impossible to apply other lifesaver therapies, such as transplant or chemotherapy.AMR is fr...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/5584_2019_399
更新日期：2019-01-01 00:00:00
abstract：:The mutational basis of APRT deficiency was studied in non-Japanese and Japanese patients. Fifteen different mutations have been identified altogether. Of these 4 were common, 6 were located in exon 3, and two at the exon 4-intron 4 junction. The common mutations were a missense mutation in exon 3 (asp65----val) and a...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4615-7703-4_16
更新日期：1991-01-01 00:00:00
abstract：:Oligomeric proteins can be intramolecularly cross-linked with polylysine in a reaction in which a water soluble carbodiimide mediates an amide linkage between the protein carboxyl groups and the epsilon-amino groups of polylysine. Studies carried out with a cytochrome p-450 indicate that a small number of molecules in...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4684-3282-4_39
更新日期：1977-01-01 00:00:00
abstract：:The synapse between the inner hair cells (IHCs) and the spiral ganglion neurons (SGNs) in mammalian cochleae is characterized as having presynaptic ribbons and therefore is called ribbon synapse. The special molecular organization is reviewed in this chapter in association with the functional feature of this synapse i...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-981-13-6123-4_3
更新日期：2019-01-01 00:00:00
abstract：:We present here an experimental approach for exploring a new class of tumor biomarkers that are overexpressed by circulating tumor cells (CTCs) and are likely targetable in immunotherapy against tumor metastasis. Using carbohydrate microarrays, anti-tumor monoclonal antibodies (mAbs) were scanned against a large panel...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-55947-6_15
更新日期：2017-01-01 00:00:00
abstract：:The effectiveness of different compounds in the generation of acrylamide (AA) from asparagine, was determined by reacting asparagine with mono-, di- and polysaccharides, as well as four different oxo-compounds known to be involved in carbohydrate metabolism/degradation. Quantitation of AA formed either under aqueous c...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/0-387-24980-x_16
更新日期：2005-01-01 00:00:00
