A Comprehensive Study of Positive Body Image as a Predictor of Psychological Well-Being in Anorexia Nervosa.

Recent data suggest a close association between positive body image (PBI) and eating disorder recovery. Nevertheless, the specific mechanisms through which PBI may facilitate recovery from anorexia nervosa (AN) remain unknown. To advance understanding of these mechanisms, this study examined core indices of PBI within AN, exploring its association with emotion regulation and well-being outcomes. Data were collected from 159 female participants, 64 with AN diagnosis and 95 healthy controls (HCs), who completed measures of PBI (body appreciation, functionality appreciation, and body responsiveness), emotion regulation, and psychological well-being (depression, anxiety, stress, and psychological quality of life). The AN group reported lower levels of PBI and psychological well-being, along with greater difficulties in regulating emotions, relative to HCs. PBI variables significantly predicted emotion regulation and psychological well-being in AN, accounting for 36% to 72% of the variance, with body appreciation emerging as the strongest predictor. These findings lend credence to the view that PBI can serve as a catalyst for psychological health. We hypothesize that enhancing PBI can improve interoceptive awareness, which is crucial for emotion regulation and reducing maladaptive food-related coping. Emphasizing a mind-body connection in lifestyle could be a relevant element to consider for both treating and preventing AN.

Clinical profiles and treatment outcomes of outpatients with interstitial lung disease and mechanic's hands: A retrospective and observational cohort.

Idiopathic inflammatory myopathies, especially antisynthetase syndrome, often appear outside of the muscles as interstitial lung disease (ILD). Another typical finding is the presence of mechanic's hands. The aim of the present study was to describe the clinical, functional, tomographic, and serological data of patients with ILD and mechanic's hands and their response to treatment and survival rates. This is a retrospective study of ILD with concurrent myopathy. Among the 119 patients initially selected, 51 had mechanic's hands. All the patients were screened for anti-Jo-1 antibodies. An expanded panel of myopathy autoantibodies was also performed in 27 individuals. Of the 51 patients, 35 had 1 or more antibodies. The most common were anti-Jo-1, anti-PL-7, and anti-PL-12, while of the associated antibodies, anti-Ro52 was present in 70% of the 27 tested individuals. A significant response to treatment was characterized by an increase in predicted forced vital capacity (FVC) of at least 5% in the last evaluation done after 6 to 24 months of treatment. A decrease in predicted FVC of at least 5%, the need for oxygen therapy, or death were all considered treatment failures. All patients were treated with corticosteroids, and 71% with mycophenolate. After 24 months, 18 patients had an increase in FVC, 11 had a decrease, and 22 remained stable. After a median follow-up of 58 months, 48 patients remained alive and three died. Patients with honeycombing on high-resolution chest tomography (log-rank = 34.65; P < .001) and a decrease in FVC ≥5% (log-rank = 18.28, P < .001) had a poorer survival rate. Patients with ILD and mechanic's hands respond well to immunosuppressive treatment.

Cancer Prevalence in Children with Inborn Errors of Immunity: Report from a Single Institution.

BACKGROUND: Inborn Errors of Immunity (IEI) comprise several genetic anomalies that affect different components of the innate and adaptive responses, predisposing to infectious diseases, autoimmunity and malignancy. Different studies, mostly in adults, have reported a higher prevalence of cancer in IEI patients. However, in part due to the rarity of most of these IEI subtypes (classified in ten categories by the Primary Immunodeficiency Committee of the International Union of Immunological Societies), it is difficult to assess the risk in a large number of patients, especially during childhood. OBJECTIVE: To document the cancer prevalence in a pediatric cohort from a single referral institution, assessing their risk, together with the type of neoplasia within each IEI subgroup. METHOD: An extensive review of clinical records from 1989 to 2022 of IEI patients who at some point developed cancer before the age of sixteen. RESULTS: Of a total of 1642 patients with IEI diagnosis, 34 developed cancer before 16 years of age, showing a prevalence (2.1%) significantly higher than that of the general age matched population (0.22). Hematologic neoplasms (mostly lymphomas) were the most frequent malignancies. CONCLUSION: This study represents one of the few reports focused exclusively in pediatric IEI cases, describing not only the increased risk of developing malignancy compared with the age matched general population (a fact that must be taken into account by immunologists during follow-up) but also the association of the different neoplasms with particular IEI subtypes, thus disclosing the possible mechanisms involved.

Are sun exposure time, dietary patterns, and vitamin D intake related to the socioeconomic status of Portuguese children?

OBJECTIVES: To investigate the association among sun exposure time, vitamin D intake, dietary patterns, and socioeconomic status in Portuguese children. METHODS: Participants aged 3-11 years (50.1% females) were recruited from public and private schools (n = 118) in the districts of Coimbra (n = 2980), Lisbon (n = 3066), and Porto (n = 2426). Parents reported their children's daily sun exposure time during the summer season and reported children's food consumption, including vitamin D food sources, using standardized questionnaires. Parents' education level was used as a proxy measure to the socioeconomic status (SES). The principal component factor analysis (PCA) method was used to identify dietary patterns. The eight dietary patterns identified were labeled in: "fast food", "rich in vitamin D", "sugary drinks", "vitamin D", "candies", "supplements", "rich in calcium" and "vegetables/healthy". Linear regression analyses were performed to investigate the association between sun exposure time and dietary patterns according to SES. RESULTS: A total of 4755 children were included. Children from high SES had significantly longer sun exposure time (p < .001) and more frequently consumed vitamin D supplements (p < .001). "Fast food" pattern showed a negative association with sun exposure time in medium and high SES (p = .014 and p < .001, respectively). CONCLUSION: Children with lower SES spend less time exposed to the sun, consume fewer dietary sources of vitamin D, and consume more foods rich in fat and sugar.

Harnessing precursor-directed biosynthesis with glucose derivatives to access cotton fibers with enhanced physical properties.

Cotton ovule in vitro cultures are a promising platform for exploring biofabrication of fibers with tailored properties. When the ovules' growth medium is supplemented with chemically synthesized cellulose precursors, it results in their integration into the developing fibers, thereby tailoring their end properties. Here, we report the feeding of synthetic glucosyl phosphate derivative, 6-deoxy-6-fluoro-glucose-1-phosphate (6F-Glc-1P) to cotton ovules growing in vitro, demonstrating the metabolic incorporation of 6F-Glc into the fibers with enhanced mechanical properties and moisture-retention capacity while emphasizing the role of molecular hierarchical architecture in defining functional characteristics and mechanical properties. This incorporation strategy bypasses the early steps of conventional metabolic pathways while broadening the range of functionalities that can be employed to customize fiber end properties. Our approach combines materials science, chemistry, and plant sciences to illustrate the innovation required to find alternative solutions for sustainable production of functional cotton fibers with enhanced and emergent properties.

Health care utilization of Hispanic/Latino veterans with epilepsy: A national population-based study.

OBJECTIVE: Hispanic/Latino people with epilepsy are a growing population that has been understudied in clinical epilepsy research. U.S. veterans are at a higher risk of epilepsy due to greater exposures including traumatic brain injury. Hispanic/Latino Veterans with Epilepsy (HL-VWEs) represent a growing population; however the treatment utilization patterns of this population have been vastly understudied. METHODS: HL-VWE were identified from administrative databases during fiscal year 2019. Variables compared between Hispanic and non-Hispanic VWEs included demographics, rurality, service era, utilization of clinical services/investigations, and service-connected injury. Chi-square and Student's t tests were used for comparisons. RESULTS: Among 56 556 VWEs, 3247 (5.7%) were HL. HL-VWEs were younger (59.2 vs 63.2 years; p < .01) and more commonly urban-dwelling (81.6% vs 63.2%, p < .01) compared to non-HL-VWEs. They were also more likely to have served in recent missions such as the Persian Gulf War and post- 9/11 wars (p < .01). HL-VWEs had a higher utilization of all neurology services examined including neurology clinic visits, computed tomography (CT) scans, magnetic resonance imaging (MRI) scans, electroencephalography (EEG), epilepsy monitoring, and comprehensive epilepsy care (p < .01 for all). HL-VWEs were more likely to visit an emergency room or have seizure-related hospitalizations (p < .01). HL-VWEs were more likely to have a service-connected disability greater or equal to 50% (p < .01). SIGNIFICANCE: This study is one of the largest cohorts examining HL-VWEs. We found higher utilization of services in neurology, epilepsy, and neuroimaging by HL-VWEs. HL-VWE are younger, more commonly urban-dwelling, and more likely to have served during recent combat periods and have higher amounts of service-connected disability. Given that the proportion of Hispanic veterans is projected to rise over time, more research is needed to provide the best interventions and mitigate the long-term impact of epilepsy on this diverse patient group.

Knowledge and Attitudes Regarding the Vaccination of Brazilian Immigrants in Portugal: Risks When Returning to Their Country of Origin?

Vaccination is one of the main advancements in public health in the prophylaxis of infectious diseases. We intend to describe the general knowledge about vaccines/vaccination among Brazilian immigrants in Portugal, characterize their attitudes toward vaccination, and describe their knowledge of the yellow fever (YF) vaccine. A cross-sectional study was conducted using a self-completion questionnaire (face-to-face or remote). A total of 542 people participated in the study; the mean age was 36.81 years; 40.1% were male; 44.8% had their 12th year of schooling; and 27.0% had resided for ≥10 years in Portugal. Regarding general knowledge about vaccination, 53.8% answered at least 6/8 questions correctly. A total of 37.1% tended to have a favorable attitude toward vaccination. Concerning traveling, 76.7% attributed the risk of disease at the destination as the main reason for accepting vaccines. A total of 89.3% knew that there was a risk of YF in Brazil. A total of 40% answered correctly only one question about the YF vaccine; 21.6% did not answer any questions correctly. Thus, most of the Brazilian immigrants in this study have high general knowledge about vaccines/vaccination, few have a favorable attitude, and their knowledge about the YF vaccine is scarce. This could limit vaccination adherence when visiting Brazil, making health education actions necessary to increase knowledge and prevent YF risks.

Assessment of Boll Weevil Susceptibility to Isocycloseram and Ethiprole and Differential Toxicity to Natural Enemies.

Insecticides efficient against the target species while conserving natural enemies in the agroecosystem are required for IPM. With the imminent discontinuation of fipronil, a broad-spectrum insecticide, ethiprole, which belongs to the same group as phenylpyrazole (2B), and isocycloseram, a novel isoxazoline insecticide with distinct mode of action (30), provide options for controlling boll weevil. The susceptibility of the boll weevil, Anthonomus grandis grandis (Boh.), and two natural enemies [Eriopis connexa (Germar) and Bracon vulgaris Ashmead] to these insecticides were studied. Furthermore, the survival and biological traits of the lady beetle, E. connexa, exposed to fipronil, isocycloseram, and ethiprole were assessed. The LC50s values for fipronil, ethiprole, and isocycloseram for A. grandis grandis were 2.71, 0.32, and 0.025 mg a.i./L, respectively; 0.86, > 200, and 3.21 mg a.i./L for E. connexa; and 2.31, 592.94, and 0.18 mg a.i./L for B. vulgaris, respectively. The recommended rates of ethiprole did not cause mortality in adult lady beetles, although fipronil and isocycloseram were highly toxic. Lady beetle larvae and adults survived more than 80% when exposed to dried residues of ethiprole, but less than 10% when exposed to fipronil and isocycloseram. Lady beetle larvae development, reproduction, and predation rates of adults were similar between ethiprole and the control group. Although fipronil and ethiprole belong to the same insecticide group, the difference in toxicity to boll weevils and natural enemies is presented and discussed. Ethiprole was more toxic to boll weevils than to its parasitoid and lady beetle, and isocycloseram was highly toxic to all three species.

The role of the human hippocampus in decision-making under uncertainty.

The role of the hippocampus in decision-making is beginning to be more understood. Because of its prospective and inferential functions, we hypothesized that it might be required specifically when decisions involve the evaluation of uncertain values. A group of individuals with autoimmune limbic encephalitis-a condition known to focally affect the hippocampus-were tested on how they evaluate reward against uncertainty compared to reward against another key attribute: physical effort. Across four experiments requiring participants to make trade-offs between reward, uncertainty and effort, patients with acute limbic encephalitis demonstrated blunted sensitivity to reward and effort whenever uncertainty was considered, despite demonstrating intact uncertainty sensitivity. By contrast, the valuation of these two attributes (reward and effort) was intact on uncertainty-free tasks. Reduced sensitivity to changes in reward under uncertainty correlated with the severity of hippocampal damage. Together, these findings provide evidence for a context-sensitive role of the hippocampus in value-based decision-making, apparent specifically under conditions of uncertainty.

Predictors of serious infections in rheumatoid arthritis-a prospective Brazilian cohort.

BACKGROUND: Infections increase mortality and morbidity and often limit immunosuppressive treatment in rheumatoid arthritis patients. OBJECTIVE: To analyze the occurrence of serious infections and the associated factors in a cohort of rheumatoid arthritis patients under real-life conditions. METHODS: We analyzed data from the REAL, a prospective observational study, that evaluated Brazilian RA patients, with clinical and laboratory data collected over a year. Univariate and multivariate analyses were performed from the adjustment of the logistic regression model Generalized Estimating Equations (GEE), with the primary outcome being the occurrence of serious infection, defined as need for hospitalization or use of intravenous antibiotics for its treatment. RESULTS: 841 patients were included with an average follow-up time of 11.2 months (SD 2.4). Eighty-nine serious infections occurred, corresponding to 13 infections per 100 patient-years. Pulmonary fibrosis, chronic kidney disease (CKD) and central nervous system disease increased the chances of serious infection by 3.2 times (95% CI: 1.5-6.9), 3.6 times (95% CI: 1.2-10.4) and 2.4 times (95% CI: 1.2-5.0), respectively. The use of corticosteroids in moderate doses increased the chances by 5.4 times (95% CI: 2.3-12.4), and for each increase of 1 unit in the health assessment questionnaire (HAQ), the chance increased 60% (95% CI: 20-120%). CONCLUSION: The use of corticosteroids at moderate doses increased the risk of serious infection in RA patients. Reduced functionality assessed by the HAQ and comorbidities were other important factors associated with serious infection in this cohort.

Should organized sport characteristics be considered as a strategy for meeting physical activity guidelines in children?

BACKGROUND: A clearer understanding of the relationships between specific sport context with overall physical activity (PA) and sedentary time (ST) may contribute to the development of more accurate preventive strategies to increase children's engagement in PA. PURPOSE: This study aimed to examine how different organized sports contributed to children's daily PA and ST. METHODS: PA was measured for seven days via accelerometers, in 410 children aged 6-10 years (49.8% boys). Of those, 332 (53.0% boys) were engaged in an organized sport and were further considered for statistical analyses. Parents reported children's sport participation (e.g. which sport, number of times per week, duration). The sports were classified into: indoor vs. outdoor; individuals vs. team; combat vs. individual aesthetic vs. racing vs. invasion. Children's height and weight were objectively collected. Multiple one-way analyses of covariance were used to examine the effects of sport characteristics on PA and ST. A linear regression, adjusted for children's sex, age, body mass index and father's educational level, determined the relationship between being involved in multiple PA and sedentary behaviours with Moderate to Vigorous PA (MVPA) levels. RESULTS: Although engaged in an organized sport, only 30% of the children achieved the PA recommendations. Sport (compared with active commute and active play) was the best contributor to daily MVPA. Outdoor sports (vs. indoor) contributed the most to vigorous PA (VPA) and MVPA. Team sports (vs. individual) were significantly associated with lower ST. Children in combat sports accumulated more VPA and MVPA, while those in racing sports showed a higher ST. CONCLUSIONS: Sport participation alone does not guarantee children will reach the PA guidelines, and the type of sport can influence children's PA levels. Gender-stereotypes in sports may prevent girls from achieving their 60 minutes of MVPA daily.

Genetic structure and diversity of the rfb locus of pathogenic species of the genus Leptospira.

Leptospirosis is caused by pathogenic strains of the genus Leptospira and is considered the most widespread zoonotic bacterial disease. The genus is characterized by the large number of serology variants, which challenges developing effective serotyping methods and vaccines with a broad spectrum. Because knowledge on the genetic basis of the serological diversity among leptospires is still limited, we aimed to explore the genetic structure and patterns of the rfb locus, which is involved in the biosynthesis of lipopolysaccharides, the major surface antigen that defines the serovar in leptospires. Here, we used genomic data of 722 pathogenic samples and compared the gene composition of their rfb locus by hierarchical clustering. Clustering analysis showed that the rfb locus gene composition is species-independent and strongly associated with the serological classification. The samples were grouped into four well-defined classes, which cluster together samples either belonging to the same serogroup or from different serogroups but sharing serological affinity. Our findings can assist in the development of new strategies based on molecular methods, which can lead to better tools for serological identification in this zoonosis.

Relative incidence of interstitial lung diseases in Brazil.

OBJECTIVE: To assess the relative frequency of incident cases of interstitial lung diseases (ILDs) in Brazil. METHODS: This was a retrospective survey of new cases of ILD in six referral centers between January of 2013 and January of 2020. The diagnosis of ILD followed the criteria suggested by international bodies or was made through multidisciplinary discussion (MDD). The condition was characterized as unclassifiable ILD when there was no specific final diagnosis following MDD or when there was disagreement between clinical, radiological, or histological data. RESULTS: The sample comprised 1,406 patients (mean age = 61 ± 14 years), and 764 (54%) were female. Of the 747 cases exposed to hypersensitivity pneumonitis (HP)-related antigens, 327 (44%) had a final diagnosis of HP. A family history of ILD was reported in 8% of cases. HRCT findings were indicative of fibrosis in 74% of cases, including honeycombing, in 21%. Relevant autoantibodies were detected in 33% of cases. Transbronchial biopsy was performed in 23% of patients, and surgical lung biopsy, in 17%. The final diagnoses were: connective tissue disease-associated ILD (in 27%), HP (in 23%), idiopathic pulmonary fibrosis (in 14%), unclassifiable ILD (in 10%), and sarcoidosis (in 6%). Diagnoses varied significantly among centers (c2 = 312.4; p < 0.001). CONCLUSIONS: Our findings show that connective tissue disease-associated ILD is the most common ILD in Brazil, followed by HP. These results highlight the need for close collaboration between pulmonologists and rheumatologists, the importance of detailed questioning of patients in regard with potential exposure to antigens, and the need for public health campaigns to stress the importance of avoiding such exposure.

Effects of salinity on pre- and post-fertilization developmental events in the mangrove oyster Crassostrea rhizophorae (GUILDING, 1828).

The mangrove oyster Crassostrea rhizophorae is identified as a potentially valuable species for tropical aquaculture, however, information on the physiological mechanisms of reproduction under laboratory conditions for this species is limited. This study investigated the effects of salinity at different concentrations (15, 20, 25, 30, 35, and 40 g/L) on the induction of germinal vesicle breakdown (GVBD) of oocytes obtained through stripping, the release of polar bodies (PB1 and PB2), and the larval development of the mangrove oyster. The results revealed a relationship between salinity and the percentage of GVBD, with the most effective range being 30-40 g/L within the hydration time frame between 70 and 120 min. The release of 50 % of PB1 was detected within this salinity range, while for the release of 50 % of PB2, the saline treatments of 35 and 40 g/L showed the best results. Overall, the salinity range of 30-40 g/L is suggested as the most suitable of polyploidy induction methodologies through the retention of PB1 or PB2. Regarding larval hatching, while salinities between 25 and 40 g/L presented similar percentages, at 15 g/L no hatching was observed. This study demonstrated that salinity is a key factor in early pre- and post-fertilization stages for the successful reproduction of mangrove oyster in hatcheries and that the percentages of oocyte maturation and artificial fertilization can be optimized by adjusting salinity.

BatFly: A database of Neotropical bat-fly interactions.

Global changes have increased the risk of emerging infectious diseases, which can be prevented or mitigated by studying host-parasite interactions, among other measures. Bats and their ectoparasitic flies of the families Streblidae and Nycteribiidae are an excellent study model but, so far, our knowledge has been restricted to fragmented records at a local scale. To help boost research, we assembled a data set of bat-fly interactions from 174 studies published between 1904 and 2022 plus three original data sets. Altogether, these studies were carried out at 650 sites in the Neotropics, mainly distributed in Mexico, Brazil, Argentina, southern USA, and Colombia, among other countries. In total, our data set contains 3984 interaction records between 237 bat species and 255 fly species. The bat species with the largest number of recorded interactions were Carollia perspicillata (357), Artibeus jamaicensis (263), and Artibeus lituratus (228). The fly species with the largest number of recorded interactions were Trichobius joblingi (256), Megistopoda aranea (235), and Megistopoda proxima (215). The interaction data were extracted, filtered, taxonomically harmonized, and made available in a tidy format together with linked data on bat population, fly population, study reference, sampling methods and geographic information from the study sites. This interconnected structure enables the expansion of information for each interaction record, encompassing where and how each interaction occurred, as well as the number of bats and flies involved. We expect BatFly to open new avenues for research focused on different levels of ecological organization and spatial scales. It will help consolidate knowledge about ecological specialization, resource distribution, pathogen transmission, and the drivers of parasite prevalence over a broad spatial range. It may also help to answer key questions such as: Are there differences in fly prevalence or mean infestation across Neotropical ecoregions? What ecological drivers explain those differences? How do specialization patterns vary among fly species in the Neotropics? Furthermore, we expect BatFly to inspire research aimed at understanding how climate and land-use changes may impact host-parasite interactions and disease outbreaks. This kind of research may help us reach Sustainable Development Goal 3, Good Health and Wellbeing, outlined by the United Nations. The data are released under a Creative Commons Attribution 4.0 International License.

Nutritional Knowledge about Maternal and Newborn Health among Physiotherapists during the COVID-19 Pandemic in Minas Gerais, Brazil.

Adequate nutrition before and during pregnancy, as well as postpartum, is among the major contributors to maternal and newborn health. Physiotherapists' knowledge of this area is still scarce, although their clinical practice has been linked to newborns' neuropsychomotor development, which, in turn, is influenced by maternal health and nutritional status. Therefore, this study aimed to evaluate the nutritional knowledge of physiotherapists regarding maternal and newborn health. A total of 70 Brazilian physiotherapists (32.2 ± 6.0 years; 72.9% females) were evaluated between November 2019 and February 2020 for their sociodemographic characteristics, professional experience, and nutritional knowledge about maternal and newborn health through a validated questionnaire personally administered by the same trained researcher. Most of the physiotherapists had graduated but had no specialization in maternal and child physiotherapy (96.1% of the females and all the males). The nutritional knowledge about maternal and newborn health was significantly different between the female and male health professionals, as well as between the less and more experienced participants, i.e., female physiotherapists and the more experienced ones had more correct answers on the nutritional questionnaire than the male and less experienced physiotherapists, respectively (p < 0.05). Our results open an interesting window for the future education and training of Brazilian physiotherapists in nutrition.

Microbial Biomineralization of Alkaline Earth Metal Carbonates on 3D-Printed Surfaces.

The biomineralizing bacterium Sporosarcina pasteurii has attracted considerable interest in the area of geotechnical engineering due to its ability to induce extracellular mineralization. The presented study investigated S. pasteurii's potential to induce the mineralization of alkali-earth metal carbonate coatings on different polymeric 3D-printed flat surfaces fabricated by different additive manufacturing methods. The use of calcium, barium, strontium, or magnesium ions as the source resulted in the formation of vaterite (CaCO3), witherite (BaCO3), strontianite (SrCO3), and nesquehonite MgCO3·3H2O, respectively. These mineral coatings generally exhibit a compact, yet variable, thickness and are composed of agglomerated microparticles similar to those formed in solution. However, the mechanism behind this clustering remains unclear. The thermal properties of these biologically induced mineral coatings differ from their inorganic counterpart, highlighting the unique characteristics imparted by the biomineralization process. This work seeks to capitalize on the bacterium S. pasteurii's ability to form an alkali-earth metal carbonate coating to expand beyond its traditional use in geoengineering applications. It lays the ground for a novel integration of biologically induced mineralization of single or multilayered and multifunctional coating materials, for example, aerospace applications.

ZNF750 Regulates Skin Barrier Function by Driving Cornified Envelope and Lipid Processing Pathways.

The epidermis is a constantly renewing stratified epithelial tissue that provides essential protective barrier functions. The major barrier is located at the outermost layers of the epidermis, formed by terminally differentiated keratinocytes reinforced by proteins of their cornified envelope and sequestered intercellular lipids. Disruptions to epidermal differentiation characterize various skin disorders. ZNF750 is an epithelial transcription factor essential for in vitro keratinocyte differentiation, whose truncating mutation in humans causes autosomal dominant psoriasis-like skin disease. In this study, we utilized an epidermal-specific Znf750 conditional knockout mouse model to uncover the role ZNF750 plays in epidermal development. We show that deletion of Znf750 in the developing skin does not block epidermal differentiation completely, suggesting in vivo compensatory feedback mechanisms, although it does result in impaired barrier function and perinatal lethality. Molecular dissection revealed ultrastructural defects in the differentiated layers of the epidermis, accompanied by alterations in the expression of ZNF750-dependent genes encoding key cornified envelope precursor proteins and lipid-processing enzymes, including gene subsets known to be mutated in human skin diseases involving impaired barrier function. Together, our findings provide molecular insights into the pathogenesis of human skin disease by linking ZNF750 to a subset of epidermal differentiation genes involved in barrier formation pathways.