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_name
Adv Exp Med Bioljournal_title
Advances in experimental medicine and biologyauthors
Larochelle Jdoi
10.1007/978-3-319-55780-9_1subject
Has Abstractpub_date
2017-01-01 00:00:00pages
3-8eissn
0065-2598issn
2214-8019journal_volume
959pub_type
杂志文章abstract：:Biochemical imbalances, provoked by aging or a secondary illness, might directly affect the brain, causing severe problems, such as loss of memory or alteration of behavior patterns. Brain disorders are usually classified as injuries (such as stroke, hematomas, and concussions), tumors, and neurodegenerative (such as ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-90143-5_2
更新日期：2018-01-01 00:00:00
abstract：:Circular RNAs characterize a class of widespread and diverse endogenous RNAs which are non-coding RNAs that are made by back-splicing events and have covalently closed loops with no polyadenylated tails. Various indications specify that circular RNAs (circRNAs) are plentiful in the human transcriptome. However, their ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-981-13-1426-1_8
更新日期：2018-01-01 00:00:00
abstract：:Fibers are broadly classified as soluble and insoluble based on their physical or analytical properties. Two human studies have been carried out in this laboratory utilizing soluble gums. The first compared a low fiber diet to the diet with an average of 19.5 g of added fiber per day from cellulose, an insoluble fiber...
journal_title：Advances in experimental medicine and biology
pub_type： 临床试验,杂志文章
doi：10.1007/978-1-4684-5784-1_2
更新日期：1990-01-01 00:00:00
abstract：:Fibrin is a major component of atherosclerotic plaques, and there may also be situations in which intravascular fibrin is formed in contact with the endothelium. The studies to be presented describe the distribution of fibrinogen/fibrin I, fibrin II, and fragments D and D-dimer in normal vessels and atherosclerotic pl...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4615-3806-6_33
更新日期：1990-01-01 00:00:00
abstract：:To assess whether the dipeptide N-epsilon-(gamma-L-glutamyl)-L-lysine (glutamyl-lysine) can serve as a nutritional source of lysine, we compared the growth of mice fed (a) an amino acid diet in which lysine was replaced by six dietary levels of glutamyl-lysine; (b) wheat gluten diets fortified with lysine; (c) a wheat...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4899-2626-5_30
更新日期：1991-01-01 00:00:00
abstract：:Our in vitro data indicate that peripheral blood monocytes or monocyte-derived immature dendritic cells under appropriate culture conditions transdifferentiate into endothelial-like cells (ELC), which are characterized by the expression of endothelial markers and the formation of tube-like structures. Dependent on the...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4615-0169-5_6
更新日期：2003-01-01 00:00:00
abstract：:Digenetic trematodes form a major group of human parasites, affecting a large number of humans, especially in endemic foci. Over 100 species have been reported infecting humans, including blood, lung, liver, and intestinal parasites. Traditionally, trematode infections have been diagnosed by parasitological methods ba...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-030-18616-6_14
更新日期：2019-01-01 00:00:00
abstract：:Marine invertebrates at the base of oceanic trophic webs play important ecological and economical roles supporting worldwide fisheries worth millions. There is an increasing concern about the effects of anthropogenic noise on marine fauna but little is known about its effects on invertebrates. Here the current peer-re...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4939-2981-8_3
更新日期：2016-01-01 00:00:00
abstract：:The origin of some non communicable disease (NCDs) is in early life. Evidence has shown that early life nutrition is associated with the risk of developing chronic non communicable diseases. Pregnancy and infancy are the most critical stages that influence the risks of NCDs in childhood and adult life. Prenatal matern...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-3-030-10616-4_4
更新日期：2019-01-01 00:00:00
abstract：:Kisspeptins are a family of overlapping neuropeptides, encoded by the Kiss1 gene, that are required for activation and maintenance of the mammalian reproductive axis. Kisspeptins act within the hypothalamus to stimulate release of gonadotrophic releasing hormone and activation of the pituitary-gonadal axis. Robust mod...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4614-6199-9_22
更新日期：2013-01-01 00:00:00
abstract：:Acute disseminated encephalomyelitis (ADEM) is a disorder of the central nervous system (CNS) characterized by an acute event, typically with encephalopathy, in which diffuse CNS involvement occurs. It may follow an infectious event and occurs more commonly in young children. Pulse steroid treatment is frequently used...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4614-0653-2_1
更新日期：2012-01-01 00:00:00
abstract：:Pertinent data from the literature and in press is summarised and used to construct a model for the molecular arrangement of lipid and protein in the lamellae of compact central nerve myelin. For the lipid phase of myelin the available data is best interpreted in terms of a bilayer arrangement while physical studies s...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4684-2514-7_17
更新日期：1978-01-01 00:00:00
abstract：:We discuss an overtly "simple approach" to complex biological systems borrowing selectively from theoretical physics. The approach is framed by three maxims, and we show examples of its success in two different applications: investigating cellular robustness at the level of gene regulatory networks and quantifying rar...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-32189-9_18
更新日期：2016-01-01 00:00:00
abstract：:Fibromyalgia (FM) is a prevalent syndrome with chronic pain and a hypothesised underlying disturbance of the tryptophan (TRP) metabolism. We performed a tryptophan depletion (TD) test in 17 FM patients and 17 controls. TRP, 5-hydroxyindoleacetic acid (5-HIAA), kynurenine (KYN), and Interleukin-6 (IL-6) were measured. ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4615-0135-0_30
更新日期：2003-01-01 00:00:00
abstract：:Sarcoidosis is a systemic granulomatous disease with unknown etiology. Lungs and lymph nodes are commonly affected. Also, cases of pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are described. However, the exact prevalence of PAH in patients with sarcoidosis is unclear. A 111 patients with prove...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-94-007-4546-9_38
更新日期：2013-01-01 00:00:00
abstract：:It is well established that palmitoylation plays a key role in the regulation of immune checkpoints, but the technical challenges in detecting protein palmitoylation have significantly prohibited further researches in this field. Till now, different approaches have been proposed, such as mutagenesis, antibody-based me...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-981-15-3266-5_17
更新日期：2020-01-01 00:00:00
abstract：:Five patients with active Wegener's granulomatosis were treated with the immunosuppressive agent Cyclosporin A, along with low dose prednisone. All five patients had previously taken cyclophosphamide, but further treatment with this agent was not desired, either due to patient choice, drug toxicity or malignancy. In i...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4757-9182-2_85
更新日期：1993-01-01 00:00:00
abstract：:Apolipoproteins are a key component of lipid transport in the circulatory system and share a number of structural features that facilitate this role. When bound to lipoprotein particles, these proteins are relatively stable. However, in the absence of lipids they display conformational instability and a propensity to ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-319-17344-3_7
更新日期：2015-01-01 00:00:00
abstract：:PLTP plays an important role in lipoprotein metabolism and cardiovascular disease development in humans; however, the mechanisms are still not completely understood. In mouse models, PLTP deficiency reduces cardiovascular disease, while its overexpression induces it. Therefore, we used mouse models to investigate the ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-981-15-6082-8_1
更新日期：2020-01-01 00:00:00
abstract：:Preserving the integrity of proteins, biomolecules prone to molecular damage, is a fundamental function of all biological systems. Impairments in protein quality control (PQC) may lead to degenerative processes, such as aging and various disorders and diseases. Fortunately, cells contain a hierarchical system of pathw...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4419-7002-2_9
更新日期：2010-01-01 00:00:00
abstract：:With the rapid advancement of high-throughput DNA sequencing technologies, genomics has become a big data discipline where large-scale genetic information of human individuals can be obtained efficiently with low cost. However, such massive amount of personal genomic data creates tremendous challenge for privacy, espe...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-981-10-6041-0_8
更新日期：2017-01-01 00:00:00
abstract：:The heat shock response (HSR) is characterized by the induction of molecular chaperones following a sudden increase in temperature. In eukaryotes, the HSR comprises the set of genes controlled by the transcription factor Hsf1. The HSR is induced by defects in co-translational protein folding, ribosome biogenesis, orga...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-030-40204-4_3
更新日期：2020-01-01 00:00:00
abstract：:Lowered concentration of Ca2+ ions, resulting from illumination of the photoreceptor cell, is the signal for resynthesis of cGMP by retina-specific guanylyl cyclase (retGC). This Ca2+-dependent activation of retGC is mediated by Ca2+-binding proteins named GCAPs (guanylyl cyclase-activating proteins) and contributes t...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4615-0121-3_19
更新日期：2002-01-01 00:00:00
abstract：:Protein folding and structure have been of interest since the dawn of protein chemistry. Following translation from the ribosome, a protein must go through various steps to become a functional member of the cellular society. Every protein has a unique function in the cell and is classified on this basis. Proteins that...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-3-319-23603-2_12
更新日期：2015-01-01 00:00:00
abstract：:In the past 12 years, since the first description of C. elegans germ cell apoptosis, this area of research rapidly expanded. It became evident that multiple genetic pathways lead to the apoptotic demise of germ cells. We are only beginning to understand how these pathways that all require the CED-9/Bcl-2, Apaf-1/CED-4...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4614-4015-4_9
更新日期：2013-01-01 00:00:00
abstract：:Asian (Elephas maximus) and African (Loxodonta africana) elephants serve as important keystone, umbrella and flagship species. Despite that, population numbers are declining, due mainly to poaching and habitat destruction. Understanding reproductive mechanisms is vital to effective management, particularly insurance p...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-3-030-23633-5_9
更新日期：2019-01-01 00:00:00
abstract：:The objective of this work was to establish a novel and robust technology, based on electron paramagnetic resonance (EPR) oximetry, as a practical tool for measurement of tumor oxygen. Previously, we have reported on the development of oxygen-sensing paramagnetic crystals (LiNc-BuO) encapsulated in a biocompatible pol...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-3-319-55231-6_41
更新日期：2017-01-01 00:00:00
abstract：:Macaca, Callithrix jacchus marmoset monkey, Pan troglodytes chimpanzee and human retinas were examined to define if short wavelength (S) cones share molecular markers with L&M cone or rod photoreceptors. S cones showed consistent differences in their immunohistochemical staining and expression levels compared to L&M c...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4614-3209-8_7
更新日期：2014-01-01 00:00:00
abstract：:Five human leukocyte long-term suspension cultures were investigated by means of cytochemical and cytogenetic methods. A striking resemblance in the morphology of these cells originating either from peripheral blood or from spleen of patients with or without hematological disorders was found. Lymphocytic origin of all...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4757-0731-1_15
更新日期：1975-01-01 00:00:00
abstract：:Many cases of West's syndrome are inoperable, because there is no clear epileptic focus. In some cases, however, there is a structural abnormality which can be surgically removed. Dr. Hoffman discusses his experience with surgery for cortical dysplasia in West's syndrome. ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：
更新日期：2002-01-01 00:00:00
