Abstract：
:Autosomal dominant retinitis pigmentosa (adRP) is mainly caused by mutations responsible for rhodopsin (RHO) misfolding. Although it was previously proved that unfolded RHO is retained into the endoplasmatic reticulum (ER) eliciting ER-stress, consequent mechanisms underlying photoreceptor degeneration need to be further clarified. Several animal models of RHO mutants have been developed for this purpose and for development of neuroprotective treatments. Here, we compared two of the most used models of adRP, the P23H mutant RHO transgenic and knock-in mouse models, in order to define which are their limits and potentials. Although they were largely used, the differences on the activation of the cell death pathways occurring in these two models still remain to be fully characterized. We present data proving that activation of calpains is a mechanism of cell death shared by both models and that molecules targeting calpains are neuroprotective. Conversely, the role of ER-stress contribution to cell death appears to be divergent and remains controversial.
journal_name
Adv Exp Med Bioljournal_title
Advances in experimental medicine and biologyauthors
Comitato A,Schiroli D,La Marca C,Marigo Vdoi
10.1007/978-3-030-27378-1_51subject
Has Abstractpub_date
2019-01-01 00:00:00pages
311-316eissn
0065-2598issn
2214-8019journal_volume
1185pub_type
杂志文章abstract：:During development in vivo and in vitro, estrogens: a) increase brain excitability, particularly in limbic structures; b) are responsible for the maturation and cyclicity of limbic-hypothalamic interrelations; c) enhance myelinogenesis; and d) may act with NGF to stimulate neurite formation. In senescence, estrogen ad...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4757-9551-6_19
更新日期：1997-01-01 00:00:00
abstract：:A collaborative study of the ampouled preparation of porcine pancreatic kininogenase, the Proposed International Reference Preparation (PIRP), was carried out, on behalf of the World Health Organization, by 19 laboratories in seven countries. The PIRP was studied using in vivo bioassays, immunoassays and in vitro enzy...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：
更新日期：1983-01-01 00:00:00
abstract：:Our own studies and those of others have shown that defects in essential fatty acid (EFA) metabolism occurs in age-related disorders such as obesity, type 2 diabetes mellitus, hypertension, atherosclerosis, coronary heart disease, immune dysfunction and cancer. It has been noted that in all these disorders there could...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-030-42667-5_3
更新日期：2020-01-01 00:00:00
abstract：:Curcumin was found to be cytotoxic in nature to a wide variety of tumor cell lines of different tissue origin. The action of curcumin is dependent on with the cell type, the concentration of curcumin (IC50: 2-40 microg/mL), and the time of the treatment. The major mechanism by which curcumin induces cytotoxicity is th...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-0-387-46401-5_6
更新日期：2007-01-01 00:00:00
abstract：:Anthropogenic sounds come in different forms, varying not only in amplitude and frequency spectrum but also in temporal structure. Although fish are sensitive to the temporal characteristics of sound, little is known about how their behavior is affected by anthropogenic sounds of different temporal patterns. We invest...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4939-2981-8_93
更新日期：2016-01-01 00:00:00
abstract：:Enteric neuropathy is a term indicating an impairment of the innervation supplying the gastrointestinal tract. The clinical phenotypes of the enteric neuropathies are the 'tip of the iceberg' of severe functional digestive diseases, such as intestinal pseudo-obstruction syndromes (e.g., chronic intestinal pseudo-obstr...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-27592-5_12
更新日期：2016-01-01 00:00:00
abstract：:Xeno-transplantation of pancreatic islets represents a promising therapeutic alternative for the treatment of type 1 diabetes mellitus. However, potent innate immune responses induced shortly after the transplantation of donor islets to the recipient, comprising the Instant Blood Mediated Immune Reaction (IBMIR), exer...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-18603-0_11
更新日期：2015-01-01 00:00:00
abstract：:The characteristic functions of relaxin are associated with female reproductive tract physiology. These include the regulation of biochemical processes involved in remodeling the extracellular matrix of the cervix and vagina during pregnancy and rupture of the fetal membranes at term. Such modifications enable the you...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-0-387-74672-2_4
更新日期：2007-01-01 00:00:00
abstract：:Exosomes are 60 to 90 nm membrane vesicles originating from late endosomes and secreted from most hematopoietic and epithelial cells in vitro. B cell derived-exosome antigenicity was first reported in 1996 in MHC class II restricted CD4+ T lymphocytes. In 1998, we reported that dendritic cell derived-exosomes are immu...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4615-0081-0_17
更新日期：2003-01-01 00:00:00
abstract：:Reactive oxygen species (ROS) likely play a role in the hypoxic ventilatory response. We determined whether hypoxic responses were influenced by alterations in cellular redox status induced by reductions in superoxide dismutase-1 (SOD-1) activity, a cytosolic anti-oxidant enzyme. Using whole-body, continuous-flow plet...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-0-387-73693-8_23
更新日期：2008-01-01 00:00:00
abstract：:Expression levels of five miRNAs (miR-19b, miR-21, miR-126, miR-141, miR-205) were measured in the plasma of healthy donors and prostate cancer patients. It was shown that miR-141 expression level efficiently discriminates early stage prostate cancer patients and correlates with the Gleason score. ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-3-319-42044-8_2
更新日期：2016-01-01 00:00:00
abstract：:The use of cell fusion has been applied to the development of immunotherapy cancer vaccines. This has typically involved the fusion of dendritic cells and tumor cells. The resultant hybrid uses the specialized antigen presentation properties supplied by the dendritic cell fusion partner to present tumor antigens, both...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-94-007-0763-4_11
更新日期：2011-01-01 00:00:00
abstract：:Cross sectional segments were removed from the uteri of pregnant and pseudopregnant mice and incubated in vitro with labeled amino acids. Proteins that were synthesized and secreted by these explants were separated by two-dimensional polyacrylamide gel electrophoresis and localized by fluorography. As expected, the ov...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4684-1297-0_12
更新日期：1987-01-01 00:00:00
abstract：:Four new triterpene saponins of the oleanan series-melilotosides I, II, III, IV and nonglycosylated soyasapogenol B have been isolated from roots of Melilotus albus Medik. (Leguminosae) and four steroid saponins were isolated from M. tauricus (Bieb.) ser. seeds. The chemical structure of the saponins and of their agly...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4613-0413-5_18
更新日期：1996-01-01 00:00:00
abstract：:Oxytocin is a nonapeptide hormone that participates in the regulation of parturition and lactation. It has also been implicated in various behaviors, such as mating and maternal, and memory. To investigate whether or not oxytocin (OT) is essential for any of these functions, we eliminated, by homologous recombination,...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4615-4871-3_30
更新日期：1998-01-01 00:00:00
abstract：:Given the interest of many people and families directly or indirectly affected by hereditary tyrosinemia (HT1), I have tried to give my view on the history of the disease from 1965 to 2015 (Fig. 1.1). ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-3-319-55780-9_1
更新日期：2017-01-01 00:00:00
abstract：:It has long been known that endogenous pyrogen, released as a result of injection of typhoid vaccine or in response to infection, produces fever and increases ACTH secretion. Recent studies have indicated that endogenous pyrogen is, at least in part, IL-1. This monokine has now been shown to activate the release of AC...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4684-5799-5_20
更新日期：1990-01-01 00:00:00
abstract：:Immune responses are controlled by the optimal balance between protective immunity and immune tolerance. T-cell receptor (TCR) signals are modulated by co-signaling molecules, which are divided into co-stimulatory and co-inhibitory molecules. By expression at the appropriate time and location, co-signaling molecules p...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-981-32-9717-3_2
更新日期：2019-01-01 00:00:00
abstract：:There has been a deluge of biological sequence data in the public domain, which makes sequence comparison one of the most fundamental computational problems in bioinformatics. The biologists routinely use pairwise alignment programs to identify similar, or more specifically, related sequences (having common ancestor)....
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4419-7046-6_30
更新日期：2011-01-01 00:00:00
abstract：:The purpose of this study was to investigate the antioxidant activities of short-neck clam water extract (SNC-WE) enriched in taurine. In the present study, the half-maximal inhibitory concentration (IC50) values of the SNC-WE for DPPH, superoxide, and alkyl radical scavenging activities determined by an electron spin...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-94-024-1079-2_82
更新日期：2017-01-01 00:00:00
abstract：:Although cell fusion is an omnipresent process in life, to date considerably less is still known about the mechanisms and the molecules being involved in this biological phenomenon in higher organisms. In Cell Fusion in Health and Disease Volume 1 international leading experts will present up-to-date overviews about t...
journal_title：Advances in experimental medicine and biology
pub_type：
doi：10.1007/978-94-007-0763-4_1
更新日期：2011-01-01 00:00:00
abstract：:Intermittent measurement of blood pressure (BP) is frequently performed in newborn babies under intensive care using an inflatable cuff encircling either the arm or leg. This paper describes work aimed at achieving continuous beat-by-beat measurement of the arterial pressure waveform by means of a finger cuff, the pre...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4613-1927-6_56
更新日期：1987-01-01 00:00:00
abstract：:Anthranilic acid (AA) has been attracted considerable attention as one of the L-tryptophan-kynurenine pathway metabolites in the central nervous system. In this study, the concentration of L-kynurenine (L-KYN) and AA in serum and CSF, and its related enzyme activities were determined in several species. In rabbits, CS...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4615-4709-9_79
更新日期：1999-01-01 00:00:00
abstract：:Terminal Transferase (TdT), Adenosine Deaminase (ADA), immunological membrane markers, cytochemical reactivity and cytogenetics were analyzed in 226 patients with ALL, AUL and AML, in 70 patients with CML and in 3 cases of Ph' positive acute leukemia presenting as ALL. TdT was tested in peripheral blood and bone marro...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4684-8929-3_24
更新日期：1982-01-01 00:00:00
abstract：UNLABELLED:Polychlorinated biphenyls (PCBs) and dioxins are environmental pollutants. Prenatally, as well as postnatally through breast feeding, large amounts are transferred from mother to the child. Formula is free of these substances. Considering their potential developmental neurotoxicity, we investigated long term...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：
更新日期：2000-01-01 00:00:00
abstract：:Relative to the role of viruses in the pathogenesis of atherosclerosis a number of questions remain to be answered. Being ubiquitous, are the herpesvirus infections the norm or a diseased state in humans and animals? In all cases studied, including MDV induced atherosclerosis, direct isolation of viruses from arterial...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4615-1957-7_9
更新日期：1995-01-01 00:00:00
abstract：:Neonatal stroke presents with features of encephalopathy and can result in significant morbidity and mortality. We investigated the cerebral metabolic and haemodynamic changes following neonatal stroke in a term infant at 24 h of life. Changes in oxidation state of cytochrome-c-oxidase (oxCCO) concentration were monit...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4939-3023-4_62
更新日期：2016-01-01 00:00:00
abstract：:Zebrafish are increasingly being used to study cancer. Almost all tumor types have been found in zebrafish. However, tumor incidence is relatively low and tumors develop late in life. Functional inactivation of tumor suppressors is a crucial step in cancer progression and more and more tumor suppressor genes are being...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-30654-4_4
更新日期：2016-01-01 00:00:00
abstract：:Since plasma is a homogenous fluid its biochemical systems need special control mechanisms to prevent their unlimited and continuous activity. In general, enzymic activity is not preformed but generated by trigger processes. Activation is then enhanced by cascade reactions. It can be controlled by enzyme inhibitors, b...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：
更新日期：1979-01-01 00:00:00
abstract：:This paper has described an animal (rodent) model of depression in which depression-like characteristics are produced by exposure of animals to stressful events that they cannot control. This model, called "stress-induced depression," appears to be mediated by stress-induced changes in brain norepinephrine (NE), and e...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4899-2064-5_33
更新日期：1988-01-01 00:00:00
