Mitochondrial Cardiomyopathies

Mitochondria are found in all nucleated human cells and perform a variety of essential functions, including the generation of cellular energy. Mitochondria are under dual genome control. Only a small fraction of their proteins are encoded by mitochondrial DNA (mtDNA) while more than 99% of them are encoded by nuclear DNA (nDNA). Mutations in mtDNA or mitochondria-related nDNA genes result in mitochondrial dysfunction leading to insufficient energy production required to meet the needs of various organs, particularly those with high energy requirements, including the central nervous system, skeletal and cardiac muscles, kidneys, liver, and endocrine system. Because cardiac muscles are one of the high energy demanding tissues, cardiac involvement occurs in mitochondrial diseases with cardiomyopathies being one of the most frequent cardiac manifestations found in these disorders. Cardiomyopathy is estimated to occur in 20-40% of children with mitochondrial diseases. Mitochondrial cardiomyopathies can vary in severity from asymptomatic status to severe manifestations including heart failure, arrhythmias, and sudden cardiac death. Hypertrophic cardiomyopathy is the most common type; however, mitochondrial cardiomyopathies might also present as dilated, restrictive, left ventricular noncompaction, and histiocytoid cardiomyopathies. Cardiomyopathies are frequent manifestations of mitochondrial diseases associated with defects in electron transport chain (ETC) complexes subunits and their assembly factors, mitochondrial tRNAs, rRNAs, ribosomal proteins, and translation factors, mtDNA maintenance, and coenzyme Q10 synthesis. Other mitochondrial diseases with cardiomyopathies include Barth syndrome, Sengers syndrome, TMEM70-related mitochondrial complex V deficiency, and Friedreich ataxia

Chromosome 15q24 microdeletion syndrome

Chromosome 15q24 microdeletion syndrome is a recently described rare microdeletion syndrome that has been reported in 19 individuals. It is characterized by growth retardation, intellectual disability, and distinct facial features including long face with high anterior hairline, hypertelorism, epicanthal folds, downslanting palpebral fissures, sparse and broad medial eyebrows, broad and/or depressed nasal bridge, small mouth, long smooth philtrum, and full lower lip. Other common findings include skeletal and digital abnormalities, genital abnormalities in males, hypotonia, behavior problems, recurrent infections, and eye problems. Other less frequent findings include hearing loss, growth hormone deficiency, hernias, and obesity. Congenital malformations, while rare, can be severe and include structural brain anomalies, cardiovascular malformations, congenital diaphragmatic hernia, intestinal atresia, imperforate anus, and myelomeningocele. Karyotypes are typically normal, and the deletions were detected in these individuals by array comparative genomic hybridization (aCGH). The deletions range in size from 1.7-6.1 Mb and usually result from nonallelic homologous recombination (NAHR) between paralogous low-copy repeats (LCRs). The majority of 15q24 deletions have breakpoints that localize to one of five LCR clusters labeled LCR15q24A, -B, -C, -D, and -E. The smallest region of overlap (SRO) spans a 1.2 Mb region between LCR15q24B to LCR15q24C. There are several candidate genes within the SRO, including CYP11A1, SEMA7A, CPLX3, ARID3B, STRA6, SIN3A and CSK, that may predispose to many of the clinical features observed in individuals with 15q24 deletion syndrome. The deletion occurred as a de novo event in all of the individuals when parents were available for testing. Parental aCGH and/or FISH studies are recommended to provide accurate genetic counseling and guidance regarding prognosis, recurrence risk, and reproductive options. Management involves a multi-disciplinary approach to care with the primary care physician and clinical geneticist playing a crucial role in providing appropriate screening, surveillance, and care for individuals with this syndrome. At the time of diagnosis, individuals should receive baseline echocardiograms, audiologic, ophthalmologic, and developmental assessments. Growth and feeding should be closely monitored. Other specialists that may be involved in the care of individuals with 15q24 deletion syndrome include immunology, endocrine, orthopedics, neurology, and urology. Chromosome 15q24 microdeletion syndrome should be differentiated from other genetic syndromes, particularly velo-cardio-facial syndrome (22q11.2 deletion syndrome), Prader-Willi syndrome, and Noonan syndrome. These conditions share some phenotypic similarity to 15q24 deletion syndrome yet have characteristic features specific to each of them that allows the clinician to distinguish between them. Molecular genetic testing and/or aCGH will be able to diagnose these conditions in the majority of individuals

Algae Essential Oils: Chemistry, Ecology, and Biological Activities

This chapter focuses on the essential oils and volatile fractions of seaweed. It includes an introduction to the essentials and volatile fractions and the main chemical classes found. This part is completed by a presentation of the fundamental aspects of biodiversity and the chemodiversity of the marine environment followed by the taxonomy and systematics of marine macroalgae. The heart of this chapter concerns the chemistry of volatile products extracted from marine algae. It reports the specificities of the marine natural products chemistry in comparison to that of terrestrial organisms. The description of volatile compounds in seaweed is divided into two parts, the first reports the common compounds identified in main volatile fractions and the second cover the specific volatile components. These include C11 hydrocarbons, sulfur compounds, and halogenated hydrocarbons. These latter are playing a very important role in communication and chemical defense. The last part includes aspects of chemical ecology and biological activities of volatile products

Effects of light quality on flowering and morphogenesis in Hyoscyamus niger L.

The present paper is concerned with bolting and morphogenesis of Hyoscyamus niger L. as reactions upon radiation in the visible spectrum.Experiments are described in which Hyoscyamus plants were exposed to light of various well defined spectral regions. The light of these spectral regions was applied at low intensity, supplementary to a short day in various colours, or at high intensity, as exclusive source of light. Special attention was paid to study the influence of these spectral regions given in the middle or at various other points of the 14-hour dark period, in combination with short days in various colours. Moreover, morphogenetic responses caused by sequences of night breaks of different spectral regions during the dark period were tested.In Hyoscyamus , stem elongation and flower bud initiation are linked, so that elongated plants normally have flower buds and rosette plants have not.A-MORPHOGENETIC EFFECTS OF BROAD BAND COLOURED IRRADIATION AT HIGH INTENSITY USED THROUGHOUT THE MAIN PHOTOPERIOD OR AT LOW INTENSITY, SUPPLEMENTARY TO SHORT PHOTOPERIODS IN VARIOUS COLOURS1. Hyoscyamus reacts as a typical long day plant. Artificial white light, increasing the duration of the light period, speeded up shooting, at an approximate critical day length between 10 and 12 hours (fig. 3 and table 1). However, stem formation in Hyoscyamus occurred in short days when 30 or even 10 mins far-red radiation was given after short basic photoperiods in various colours of light (figs. 10, 13).2. Hyoscyamus plants, grown in long days of blue light produced stems while plants in green and red long days remained in the rosette stage (table 2). Blue light was also more elongative than white light. Plants in blue and white light had higher top/root ratios than plants exposed to green and red light (table 2). A large portion of the top/root differences in the various light qualities was due to differences in root growth, blue and white light reducing it, and the other colours stimulating it. Moreover, strong elongation of petioles occurred as a result of exposure to blue light, whereas this elongation was absent in green and red wavelength bands, and in white light.3. Hyoscyamus reacted to supplementary irradiation in the blue and far-red regions with a marked elongation of stems (fig. 4) and petioles (fig. 6). This result indicated that in Hyoscyamus the high energy mechanism of the B-FR reaction exist. Plants grown in red light remained in the rosette stage, while additional far- red annihilated this inhibitive effect of red light (fig. 4). Moreover, 2 hrs blue light given after long days in red light (14 hrs) caused bolting, but obviously more slowly than a far-red supplement. Therefore, blue light resembled far-red in its influence but appeared less effective.4. Red supplementary light, after a short day in white light was more active than far-red under similar conditions (figs. 7, 18). This observation, however, is in contrast to the available literature which indicates that red extensions mostly are inactive. This contradiction may be due to the fact that the white light used for the main photoperiod contains a relatively high fraction of far-red. This result, and also the long-day effect observed when 6 hrs of red light were given after a short day in blue light (fig. 7) suggest that induction in Hyoscyamus requires intermediate P fr -levels. Additionally the fact that plants in long days red light remain vegetative, supports the idea that high concentrations of P fr are inhibitive.5. Antagonism between red and far-red was not observed under the experimental conditions (figs. 18, 19, 20). Generally, in far-red followed by red extensions and in red followed by far-red extensions, the two colours increased each other in producing the effect, when they were given after a short photoperiod in white light. On the other hand, daylength extensions of blue light followed by red caused a long day effect more or less equal to that of extensions by red light alone (figs. 18, 19). In this case, blue irradiation acts as darkness and red light after blue acts as a night break.B-MORPHOGENETIC EFFECTS OF VARIOUS PATTERNS OF NIGHT BREAKS GIVEN IN THE MIDDLE OR AT VARIOUS OTHER POINTS OF THE DARK PERIOD1. The colour of the basic photoperiod in conjunction with a red night interruption exerts an important influence on the photoperiodic and formative responses. Short photoperiods, rich in far-red (figs. 22, 24, 25, 38) or blue irradiation (fig. 38), combined with red night interruptions yielded quick bolting. Increase of the light intensities applied as night interruption resulted in still earlier flowering. At the intensity of red light of 1000 ergs/cm 2/sec, only 120 mins night breaks were found elongative, in combination with a main photoperiod in red light. With the light intensity of the red night breaks increased to 3800 ergs/cm 2/sec, even a 3 mins exposure led to flowering.2. A 10 mins red night break combined with white light rich in far-red (figs. 22, 24, 25) was sufficient to induce elongation and flower bud formation. The longer the duration of the red irradiation, the sooner stem elongation occurred. However, the photoperiodic response was saturated at 3 mins red night breaks in combination with the blue or mixed (R + FR) basic light periods (figs. 38, 39), and, therefore no clear-cut photoperiodic differences existed in the series of red night break duration or intensities. Generally, upon red night interruption, plants under a main light period consisting of a mixture of red and farred radiation showed the highest stems, the red ones the shortest, while the blue ones were intermediate (fig. 39). Upon a red night interruption, there was a similarity between the blue and red + far-red irradiations when used as basic short periods, the former, however, was slower than the latter.3. Far-red night interruptions (figs. 22, 24, 25 40), were promotive in most cases in durations of at least 10 mins, while increasing the duration enhanced the flowering reaction, but the reaction was slower than that upon red night breaks. However, far-red interruptions caused faster shooting than red night breaks in combination with short days in red light. The difference in far-red intensity applied for night interruptions had no important effect.4. It was observed that with increased duration of the night breaks, stem elongation occurred more rapidly, and consequently the number of leaves also were nicely correlated herewith (figs. 22, 23).5. No distinct differences in petiole length occurred as a result of the various durations of red night breaks as compared with their controls, when these night breaks were combined with red or R + FR basic periods. The only difference was that petiole length was greater with the mixed (R + FR) basic periods than with the red one. However, petiole lengths increased markedly owing to far-red night breaks as compared with their controls under the basic periods of red light and of the mixture of red and far-red light, and stronger so with the mixture than with red light. With the blue main light period, it was found that either red or far-red night breaks produced a sharp reduction of the petiole length.6. Red and far-red night breaks showed opposite photoperiodic response curves when applied at various moments during the long dark period (figs. 27, 28, 30, 33, 43, 44, table 3), red light being most active in the second part of the night, far-red in the first part. A mixture of these two radiations showed the red action curve, but on a higher level than the red itself (table 3).7. Long durations of blue light as a night break at high intensity (3800 ergs/cm 2/sec) were elongative mainly as red (fig. 43) when applied at various moments of the night, while the time curve of the action as spread over the night does not exclude the possibility of some far-red activity in the first half of the night. Short periods (10 mins) at high intensity (fig. 45) or long durations (2 hrs) at low intensity (1000 ergs/cm 2/sec) blue light failed to induce shooting (fig. 27).8. It was demonstrated that in combination with mixed light periods, red night interruptions caused rapid shooting when applied at each point of darkness even immediately after or before the basic light period without the requirement of a dark intercalation (figs. 43, 44, 45). In contrast, with red basic light periods sufficiently long dark periods must precede (at least 6 hrs of the 14-hour darkness) or follow (at least 2 hrs) the night interruption. This observation seems in agreement with the data in fig. 27, where two white light sources were used as a short basic light period; one of them contains more far-red (TL/IL) than the other (TL). In the latter case, 2 hrs of red light given either immediately after or before the main photoperiod were ineffective, only red light given later at several points during the dark period was effective for stem elongation and flower bud formation. However, in combination with the mixed white light source, red light was active at each point during the long dark period, even immediately after or before the basic light period. Additional experiments with variable short lengths of dark periods immediately before the main light period, showed that, for bolting in Hyoscyamus , it was necessary to intercalate at least some 30 mins darkness between a red night interruption and the main photoperiod in case the latter is rich in red (the TL-source) (fig. 35).C-PHOTOPERIODIC RESPONSES CAUSED BY SEQUENCES OF NIGHT BREAKS IN THE DARK PERIOD1. The data did not demonstrate antagonism between red and far-red irradiation during the night in combination with artificial white light sources (figs. 30, 31, 34, table 3). Far-red irradiation given after red night breaks enhanced the promotive action of red light, speeding up bolting particularly in the first half of the night. However, the application of red and far-red simultaneously during breaks in the long night had more effect on stem lengthening than far-red after a red night break The explanation seems to be that, since the durations of either red or far-red were long (2 hrs), far-red radiation given after a red night break could no more annihilate the flowering promotion of red light.2. Ten minutes far-red night interruption applied at various points of the night were inactive (fig. 45) with main light periods rich in far-red (R + FR) or not (R). However, 10 mins red night breaks at the same points were promotive for stem elongation in combination with the mixed basic light period (R+ FR); in case of the red basic period, red night breaks were only active at the 8th and 10th hour point of the 14-hour dark period.3. When Hyoscyamus plants were irradiated successively with 10 mins red and 10 mins far-red radiation at various points of the dark period, it seemed that far-red radiation reduced the elongative effect of the red night interruption in combination with the mixed (R + FR) basic period. This seems a partial manifestation of the antagonistic action of far-red against red with respect to the phytochrome system, however, the elongative action of red light observed was not much reduced (fig. 45). That some reduction of the red light effect was observed here, in contradiction to section 1 above, may be due to the fact that light periods were much shorter (viz., 10 mins v. 2 hrs).In connection with the red light basic period, far-red radiation given after various times of darkness was inactive, and red interruptions at some points also However, especially in the first half of the dark period, the succession of 10 mins red and 10 mins far-red led to flowering at all points of the dark period (fig. 45).Thus, reversibility of red action by subsequent far-red radiation in general, viz., except in first part of this section was not found in our experiments.Generally, the whole of our experiments with Hyoscyamus niger L. leads to the following conclusions:1. In general, applications of red and far-red reinforce each other, and the application of their mixture is particularly effective.2. The observed promotive action of blue light throughout the photoperiod or during the night appears to fit well together with the above results.3. The activity of Hyosyamus plants to red and far-red is different throughout the night viz., greater to far-red in the first half and to red in the second half.4. Speculating about the internal mechanism by which the observed reactions can be provisionally understood, it seems that those mentioned under item 1 may be explained in terms of phytochrome reactions by assuming the requirement of intermediate P fr -levels or specific ratios between P fr and P r for optimum induction.The blue light effects (item 2) fit well in this suggestion as both forms of phytochrome have a definite absorption in the blue region.The observation of the changes in sensitivity during the night (item 3) reveals the existence of some 'endogenous rhythm' which possibly might be understood as causing different changes in P r - and P fr -levels. at various points of a long dark period, or bringing about changes in the plant's sensitivity towards phytochrome stimulation.Direct evidence as to phytochrome reactions under the conditions of our experiments so far is not available but appears to form an extremely important object for future research

L’intelligence artificielle au cœur du processus de recrutementE-Recrutement 4.0 au Maroc Etat des lieux et perspectives

Depuis le 18 ème siècle, le monde a connu trois révolutions industrielles. La quatrième révolution industrielle ou l’industrie 4.0 est actuellement en pleine croissance. Cette révolution 4.0 se base énormément sur l’insertion de l’intelligence artificielle dans tous les domaines d’activité. La GRH est l’un des domaines qui a été très impacté par l’IA notamment la phase de recrutement, d’où la naissance du e-recrutement 4.0. Au Maroc, l’e- recrutement 4.0 n’est pas appliqué à 100%.Cependant les chances de réussirson insertion au sein des entreprises et des établissements publics dépendent de la volonté et l’accord du candidat marocain pour être évalué par un robot. Le présent article est articulé autour de trois points principaux ,dans un premier temps nous allons cerner la notion du e-recrutement4.0 ,présenter les principaux axes de recrutement touchés par l’intelligence artificielle et ensuite souligner les avantages et les inconvénients du e-recrutement 4.0, dans un deuxième temps nous allons décrire la situation de déséquilibre de l’offre et la demande en matière d’emploi au Maroc et démontrer la nécessité d’adopter la nouvelle forme de recrutement 4.0 afin de corriger ce déséquilibre, et finalement nous allons établir un questionnaire destiné à 380 candidats en recherche d’emploi afin d’avoir une idée de la perception des demandeurs d’emploi marocains sur l’insertion de l’intelligence artificielle au sein du processus de recrutement

La performance bancaire impactée par la digitalisation : une étude des banques marocaines

In 2023, Morocco's Internet penetration rate will reach 88.1%, compared with 84.1% the previous year. The indicator expresses the percentage of the population using the Internet. In recent years, the number of Internet users in Morocco has increased overall. This accelerated pace of Internet penetration in Morocco has opened up new opportunities for all businesses, and particularly for the banking sector. Banks are now using the electronic mode to provide services like never before. They offer a range of digital services from opening a bank account to applying for a loan using the Internet. Studies on digitization or online banking have mainly been limited to the aspect of making bank customers satisfied online. Some found that online banking had a positive impact on customer satisfaction, such as Belvaux and Notebaert in 2015, while others pointed out that customers preferred to visit branches rather than use online services, the resistant, according to Faran in 2018. The authors believe that there is a dearth of literature on the effects of digitization on bank performance. This paper aims to fill this gap by investigating the effects of digitization on bank performance through interviews with 39 employees from 13 banks. To analyze bank performance, three factors are examined: efficiency, cost effectiveness and challenges. Digitization was found to be positively associated with all three study variables. It has improved banks' performance and enabled them to move from basic banking services to comprehensive financial solutions, However, it should be noted that this study is based on interviews with a specific sample of bank employees, which may limit the generalisability of the findings to the banking industry as a whole. Furthermore, the research focuses on the Moroccan context, which could influence the transferability of the findings to other geographical and cultural contexts. To obtain a more in-depth and comprehensive understanding of the effects of digitalisation on bank performance, future research could broaden the sample and consider additional aspects.   Key words: digitization, banking, performance, internet JEL Classification: G21, G24, M15, N27, O3 Paper type: empirical articleEn 2023, le taux de pénétration de l'internet au Maroc atteindra 88,1 %, contre 84,1 % l'année précédente. L'indicateur exprime le pourcentage du peuple qui utilise l'internet. Ces dernières années, le nombre d'utilisateurs d'internet a globalement augmenté au Maroc. Ce rythme accéléré de pénétration de l'internet au Maroc a ouvert de nouvelles opportunités généralement pour toutes les entreprises et particulièrement pour le secteur bancaire. Les banques utilisent désormais le mode électronique pour fournir des services comme jamais auparavant. Elles offrent une gamme de services numériques allant de l'ouverture d'un compte bancaire à la demande d'un prêt en utilisant l'internet. Les études relatives à la numérisation ou à la banque en ligne se sont principalement limitées à l'aspect de rendre les clients de la banque satisfaits en ligne. Certaines ont constaté que les services bancaires en ligne avaient un impact positif sur la satisfaction des clients, telles que Belvaux et Notebaert en 2015, tandis que d'autres ont souligné que les clients préféraient se rendre dans les agences plutôt que de recourir aux services en ligne, les résistants, selon Faran en 2018. Les auteurs estiment une pénurie de littérature relative aux effets de la numérisation sur les performances des banques. Le présent document vise à combler cette lacune en étudiant les effets de la numérisation sur la performance des banques à l'aide d'entretiens menés auprès de 17 employés de 13 banques. Afin d'analyser la performance des banques, trois facteurs sont étudiés, à savoir l'efficacité, le coût, la rentabilité et les défis. La numérisation s'est avérée positivement associée aux trois variables de l'étude. Elle a amélioré les performances des banques et leur a permis de passer des services bancaires de base à des solutions financières complètes. Cependant, il convient de noter que cette étude repose sur des entretiens menés auprès d'un échantillon spécifique d'employés de banques, ce qui pourrait limiter la généralisation des résultats à l'ensemble de l'industrie bancaire. De plus, la recherche se concentre sur le contexte marocain, ce qui pourrait influencer la transférabilité des conclusions dans d'autres contextes géographiques et culturels. Pour obtenir une compréhension plus approfondie et complète des effets de la numérisation sur la performance des banques, des recherches futures pourraient élargir l'échantillon et considérer des aspects supplémentaires.   Mots clés : digitalisation, Banque, Performance, internet Classification JEL : G21, G24, M15, N27, O3 Type de l’article : article empirique &nbsp

LE FINANCEMENT DES ENTREPRISES INNOVANTES DE LA LITTERATURE VERS UNE ANALYSE SWOT

Les entreprises innovantes jouent un rôle décisif dans le processus de transformation des connaissances scientifiques en innovations industrielles. Leurs logiques de fonctionnement, ainsi que la spécificité des problèmes qu’elles rencontrent lors des différentes phases de développement s’expliquent aussi bien par des raisons culturelles et sociales que par des questions managériales et financières. Ces entreprises dites innovantes présentent des opportunités d’investissement risquées ce qui explique le recours à l’autofinancement. Par contre, les entreprises qui ne disposent pas de capacité de financement interne suffisante sont contraintes de rechercher du financement externe. L’objet de ce travail est de répondre à la problématique de financement des entreprises innovantes à travers une analyse SWOT (Strengths, Weaknesses, Opportunities and Threats) des différents enseignements de la littérature sur le financement interne, l’endettement bancaire ou le financement par capitaux externes

Impact du Digital sur la Performance Financière des Banques

Actuellement, la transformation digitale agit de manière considérable sur l’accroissement du secteur bancaire. Par conséquent, les banques sont amenées à faire preuve d’efficacité et de fournir un maximum d’efforts afin d’assurer une relation continue avec leurs clients, tout en dégageant un seuil de rentabilité optimum. A travers ce travail, nous allons mettre le point sur le phénomène de la digitalisation et son impact dans la détermination de la performance financière des banques et ce, selon trois points principaux : 1- Le concept de la digitalisation et son développement historique ; 2- Le concept de la performance et son mode d’analyse au niveau du secteur bancaire 3- Le lien de causes à effets entre la digitalisation et la performance bancaire. La conclusion à déduire de cette analyse est que l’utilisation du digital est un élément majeur pour promouvoir l’inclusion financière, atteindre la satisfaction et la fidélisation client, avoir une veille stratégique, et organisationnelle et surtout atteindre le niveau de performance financière souhaité