Amino Acid Deficiency Research Articles

Procollagen C-Proteinase Enhancer-1 (PCPE-1) deficiency in mice reduces liver fibrosis but not NASH progression.

Background and aimsNonalcoholic Steatohepatitis (NASH) is a major cause of end-stage liver diseases such as cirrhosis and hepatocellular carcinoma resulting ultimately in increased liver-related mortality. Fibrosis is the main driver of mortality in NASH. Procollagen C-Proteinase Enhancer-1 (PCPE-1) plays a key role in procollagen maturation and collagen fibril formation. To assess its role in liver fibrosis and NASH progression, knock-out mice were evaluated in a dietary NASH model.MethodsGlobal constitutive Pcolce-/- and WT male mice were fed with a Choline Deficient Amino acid defined High Fat Diet (CDA HFD) for 8 weeks. Liver triglycerides, steatosis, inflammation and fibrosis were assessed at histological, biochemical and gene expression levels. In addition, human liver samples from control and NASH patients were used to evaluate the expression of PCPE-1 at both mRNA and protein levels.ResultsPcolce gene deficiency prevented diet-induced liver enlargement but not liver dysfunction. Furthermore, liver triglycerides, steatosis and inflammation were not modified in Pcolce-/- male mice compared to WT under CDA HFD. However, a significant decrease in liver fibrosis was observed in Pcolce-/- mice compared to WT under NASH diet, associated with a decrease in total and insoluble collagen content without any significant modifications in the expression of genes involved in fibrosis and extracellular matrix remodeling. Finally, PCPE-1 protein expression was increased in cirrhotic liver samples from both NASH and Hepatitis C patients.ConclusionsPcolce deficiency limits fibrosis but not NASH progression in CDA HFD fed mice.

A versatile enrichment of functionalized calixarene as a facile sensor for amino acids.

Amino acids are the most important part of the human biological system due to their role in living processes. The role of amino acids stretches beyond their traditional role as a building block for proteins, and deficiency of amino acids could lead to decreased immunity, digestive problems, depression, fertility issues, lower mental alertness, slowed growth in children, and many other health issues. The acute detection of amino acids is necessary to determine the human health domain. Here, in this review, we summarize and study the calixarenes as complexes that are of immeasurable value and their utilization for amino acid detection. Key factors such as noncovalent forces, limit of detection, and the supramolecular chemistry of calixarenes with amino acids have been well described. This study presents the most recent efforts made towards the development of potential and highly efficient calixarene-based sensors for the detection of amino acids.

Effects of different artificial diets on commercial honey bee colony performance, health biomarkers, and gut microbiota

BackgroundHoney bee colonies managed for agricultural pollination are highly dependent on human inputs, especially for disease control and supplemental nutrition. Hives are routinely fed artificial “pollen substitute” diets to compensate for insufficient nutritional forage in the environment. The aim of this study was to investigate the effects of different artificial diets in a northern California, US commercial beekeeping operation from August through February. This time period represents an extended forage dearth when supplemental nutrition is used to stimulate late winter colony growth prior to almond pollination in the early spring. A total of 144 honey bee colonies were divided into 8 feeding groups that were replicated at three apiary sites. Feeding groups received commercial diets (Global, Ultra Bee, Bulk Soft, MegaBee, AP23, Healthy Bees), a beekeeper-formulated diet (Homebrew), or a sugar negative control. Diets were analyzed for macronutrient and amino acid content then evaluated with respect to honey bee colony population size, average bee weight, nutrition-related gene expression, gut microbiota abundance, and pathogen levels.ResultsReplicated at three apiary sites, two pollen-containing diets (Global and Homebrew) produced the largest colonies and the heaviest bees per colony. Two diets (Bulk Soft and AP23) that did not contain pollen led to significantly larger colonies than a sugar negative control diet. Diet macronutrient content was not correlated with colony size or health biomarkers. The sum of dietary essential amino acid deficiencies relative to leucine content were correlated with average bee weight in November and colony size used for almond pollination in February. Nutrition-related gene expression, gut microbiota, and pathogen levels were influenced by apiary site, which overrode some diet effects. Regarding microbiota, diet had a significant impact on the abundance of Bifidobacterium and Gilliamella and trended towards effects on other prominent bee gut taxa.ConclusionsMultiple colony and individual bee measures are necessary to test diet efficacy since honey bee nutritional responses are complex to evaluate. Balancing essential amino acid content relative to leucine instead of tryptophan may improve diet protein efficiency ratios. Optimization of bee diets could improve feed sustainability and agricultural pollination efficiency by supporting larger, healthier honey bee colonies.

Dietary supplements based on modified and enriched with amino acids zeolite in the cultivation of young turkeys

Relevance. Modern turkey crosses have a genetically determined high growth rate. These farm birds are very sensitive to even minor fluctuations in their diet, are susceptible to a deficiency of amino acids, protein, mineral elements and vitamins, which can contribute to a decrease in their productivity, the development of pathological processes and diseases. The use of modern technologies and scientific achievements has made it possible to prepare a new product — modified zeolite. This natural mineral is already free of impurities and microbes, with open pore windows, has a high ability to selectively exchange, and is obtained by various methods of activating zeolite in industrial conditions. At the same time, Ulyanovsk specialists have mastered the technology of enriching modified zeolite with useful substances.Methods. The aim of the work was to study the effect on the body of turkeys and the level of their productivity of additives based on modified zeolite enriched with amino acids. The experiment was organized for 60 days on 250 turkeys of the breed Hybrid Cread-mayker aged 55–60 days in the conditions of the Ulyanovsk region. The control group received the main ration (MR), the experimental group was given once a day in addition to the MR in the morning feeding in a mixture with mixed feed 5% additives of modified zeolite enriched with amino acids.Results. The inclusion of a supplement based on modified zeolite enriched with amino acids in the diet of turkeys does not have a negative effect on the hematological parameters of their blood, all indicators corresponded to the physiological norm. During the experiment in the 2nd group, the live weight of poultry was obtained larger by 8.70%, which amounted to 12,296 kg, respectively, more profit was obtained from the sale of turkey meat, total revenue was 133,210, and additional profit was 27,960 rubles, 2.41 rubles of profit was received for 1 ruble of costs. Тhe data indicate the normalization of mineral homeostasis in the body of turkeys and better absorption of nutrients in the feed diet.

Квашиоркор: патофизиологические аспекты и пути решения проблемы

Kwashiorkor is a form of severe protein-energy malnutrition known since 1995. It is observed worldwide in regions with high levels of hunger and poverty, especially where the diet consists primarily of carbohydrate-rich foods. This pathology has been reported among hundreds of thousands of children in the world. The clinical presentation of kwashiorkor resembles the symptoms of essential amino acid deficiency, which suggests that not only protein malnutrition, but also disorders of the methionine cycle take part in the pathogenesis of the disease. The study of capillary ultrastructure has shown that edema is the result of increased permeability of microvessels to protein macromolecules, including albumin. Plasma proteins are usually retained in the intravascular space by the endothelial glycocalyx, damage to which leads to edema. Oxidative stress contributes to lower levels of vitamin E and glutathione. An imbalance between the levels of reactive oxygen species and antioxidants leads to mitochondrial damage, which reduces ATP production and impairs liver function. Mitochondrial dysfunction and ATP depletion together with nutrient deficiency may contribute to the development of multiple organ dysfunction syndrome. Three clinical parameters are used to assess the severity of kwashiorkor: mid-arm muscle circumference, body mass index, and the presence of localized symmetric edema. In the absence of timely treatment in the early stages, children may have serious complications, including shock, coma, physical and mental disorders. Normalization of physiological and metabolic functions in patients with kwashiorkor is a complex task. The three-step approach to treating children with severe protein-energy malnutrition recommended by the World Health Organization includes stabilization, rehabilitation, and recurrence prevention. The prognosis in the absence of treatment is unfavorable. Key words: protein-energy malnutrition, kwashiorkor, children

Mixotrophic cultivation of Galdieria sulphuraria for C-phycocyanin and protein production

G. sulphuraria is a polyextremophilic microalga that can tolerate low pH, high temperature and high osmotic pressure. We cultivated G. sulphuraria ACUF 064 in chemostat at a biomass concentration of 134 to 243 g·m−2 aiming for maximal pigment content without compromising biomass productivity. Autotrophy was compared to ‘oxygen balanced’ mixotrophy with intracellular recirculation of oxygen and carbon dioxide. No differences were found in C-phycocyanin (C-PC) and protein content between autotrophic and mixotrophic cultures. In mixotrophy the biomass productivity and concentration were doubled compared to the photoautotrophic counterpart. In mixotrophy aeration was not needed and 89% of the substrate carbon was converted into biomass.Mixotrophically grown biomass contained 10% w/w C-PC which, combined with its high areal biomass productivity (49 g·m−2·day−1), sums up as one of the highest C-PC areal productivities ever reported (5 g·m−2·day−1) under 24 h/24 h illumination. C-PC extracted from G. sulphuraria was more stable than the currently used C-PC extracts from Spirulina. No significant loss of color was observed down to a pH of 3 and up to a temperature of 55 °C. G. sulphuraria had a protein content of 62% w/w and compared favorably with FAO dietary recommendation of adults regarding amino acid composition. G. sulphuraria contains a high proportion of essential, sulfur amino acids compared to Chlorella, Spirulina and soybean protein.Due to its attractive amino acid profile and high protein content, G. sulphuraria is a good candidate for food and feed applications to overcome sulfur amino acid deficiencies. In addition, oxygen balanced mixotrophy allows for efficient and productive cultivation of G. sulphuraria biomass.

Food proteins from animals and plants: Differences in the nutritional and functional properties

BackgroundAnimals and plants are the main sources of dietary proteins, and there are important differences in the type of protein that they supply. The differences include molecular structure, amino acid profile, digestibility, and technical functionality in food, i.e. the ability to gel, emulsify, bind water etc. These inherent differences influence their bioavailability from a human nutrition perspective, as well as the sensory quality of foods containing animal or plant proteins. These fundamental differences mean that designing plant-based foods to mimic animal foods requires much more than simple substitution of one ingredient with another. Scope and approachWe survey some of the nutritional and technological functionality data for animal- and plant-derived food proteins and discuss the nature and implications of the differences between them. Key findings and conclusionsPlant-based foods typically provide less complete protein nutrition because of lower digestibility and source-specific deficiencies in essential amino acids, compared with animal proteins. Such differences may not be as essential for adults as they are for infants and young children, due to their developmental requirements. Plant proteins can be subjected to various processes to bring their functionality closer to that of animal proteins (e.g. hydrolysis to improve solubility), but some processes that improve functionality also diminish amino acid bioaccessibility or bioactivity, creating negative nutritional consequences. Much more research and innovation are required to enhance the potential of plant proteins. In the short to medium term, nutritional and functional synergies between plant and animal proteins may offer a path to creating nutritious and attractive foods.

Nutritional risks among not exclusively breastfed infants in the first 6 months of life.

Breast milk is the ideal food for infants and its benefits can be observed in the short and long term. In crisis situations, breastfeeding should be promoted the most because it is one of the most cost-effective interventions aimed at reducing infant morbidity and mortality. In addition to the multiple advantages of breast milk in the mother-child bond and biological and immune properties, the most relevant characteristic of breast milk is that it covers all nutritional needs. When breastfeeding is not possible, the World Health Organization recommends infant formula as the first option. The second option is diluted cow's milk, which entails the risk for nutritional deficiency that should be strictly monitored and timely resolved. When infants are fed with diluted cow's milk, they are mainly at risk for iron, zinc, vitamin A, D, C, and E, amino acid and essential fatty acid deficiency.

Highlights from Recent Cancer Literature

Highlights from Recent Cancer Literature

The metabolic growth limitations of petite cells lacking the mitochondrial genome.

Eukaryotic cells can survive the loss of their mitochondrial genome, but consequently suffer from severe growth defects. 'Petite yeasts', characterized by mitochondrial genome loss, are instrumental for studying mitochondrial function and physiology. However, the molecular cause of their reduced growth rate remains an open question. Here we show that petite cells suffer from an insufficient capacity to synthesize glutamate, glutamine, leucine and arginine, negatively impacting their growth. Using a combination of molecular genetics and omics approaches, we demonstrate the evolution of fast growth overcomes these amino acid deficiencies, by alleviating a perturbation in mitochondrial iron metabolism and by restoring a defect in the mitochondrial tricarboxylic acid cycle, caused by aconitase inhibition. Our results hence explain the slow growth of mitochondrial genome-deficient cells with a partial auxotrophy in four amino acids that results from distorted iron metabolism and an inhibited tricarboxylic acid cycle.

Optimal composition of poultry balls based on natural brown flax bran

The paper established that depending on the number of natural brown flax bran and squash the prefabricated poultry meat does not significantly change its indicators, but the most optimal is sample No. 2 – with the replacement of the wheat bread norm with 50% natural brown flax bran; with the replacement of the water norm with 50% squash – Kurazh. The content of amino acids in Kurazh balls was higher than in the control sample, which indicates their biological value and quality. The study of the content of such acute deficient amino acids as lysine, methionine and tryptophan showed that the Kurage balls exceed the control sample in amino acids: lysine – by 77.6%, methionine – by 125.5%, tryptophane – by 78.1, respectively. A new kind of balls with functional ingredients has a number of useful properties for the human body and may be recommended for gerodietetic nutrition.

Functional Amino Acids and Autophagy: Diverse Signal Transduction and Application.

Functional amino acids provide great potential for treating autophagy-related diseases by regulating autophagy. The purpose of the autophagy process is to remove unwanted cellular contents and to recycle nutrients, which is controlled by many factors. Disordered autophagy has been reported to be associated with various diseases, such as cancer, neurodegeneration, aging, and obesity. Autophagy cannot be directly controlled and dynamic amino acid levels are sufficient to regulate autophagy. To date, arginine, leucine, glutamine, and methionine are widely reported functional amino acids that regulate autophagy. As a signal relay station, mammalian target of rapamycin complex 1 (mTORC1) turns various amino acid signals into autophagy signaling pathways for functional amino acids. Deficiency or supplementation of functional amino acids can immediately regulate autophagy and is associated with autophagy-related disease. This review summarizes the mechanisms currently involved in autophagy and amino acid sensing, diverse signal transduction among functional amino acids and autophagy, and the therapeutic appeal of amino acids to autophagy-related diseases. We aim to provide a comprehensive overview of the mechanisms of amino acid regulation of autophagy and the role of functional amino acids in clinical autophagy-related diseases and to further convert these mechanisms into feasible therapeutic applications.

Short-term amino acid, clinicopathologic, and echocardiographic findings in healthy dogs fed a commercial plant-based diet.

Consumer demand for commercially prepared plant-based (PB) dog food is increasing, but studies evaluating the short- or long-term effects of PB diets on canine health are lacking. The objective of this study was to assess the short-term amino acid (AA), clinicopathologic, and echocardiographic findings in 34 client-owned dogs fed a commercial extruded plant-based diet (PBD) in which pea protein was the primary protein source and 4 control dogs fed a commercial extruded traditional diet (TD). Plasma AA and whole blood taurine concentrations were measured in dogs at baseline and after 4 weeks on the PBD or the TD. Hematologic, serum biochemical, and echocardiographic testing were performed at baseline and after 12 weeks on the PBD or the TD. Four dogs in the PBD group did not complete the study. All essential AAs, except methionine, were higher in dogs after 4 weeks on the PBD compared to baseline. Taurine (plasma and whole blood) was also higher after 4 weeks on the PBD compared to baseline. A meaningful difference was detected in whole blood taurine between the PBD group and the control group at 4 weeks (P = .026) with the PBD group being higher. Median hematologic and biochemical results for the PBD group were within normal limits at baseline and at 12 weeks. In the PBD group, left ventricular internal diastolic dimension (LVIDd, P = < .001) and normalized LVIDd (P = .031) were higher 12 weeks post-PBD compared to baseline. There were no meaningful differences in left ventricular internal systolic dimension (LVIDs), normalized LVIDs, or fractional shortening 12 weeks post-PBD. There was no statistical evidence of difference between the 2 groups of dogs for any of the echocardiographic parameters at baseline or at 12 weeks. Essential AA or taurine deficiency was not observed in this cohort of dogs fed a commercial extruded PBD. Additionally, clinically relevant hematologic, serum biochemical and echocardiographic alterations were not detected. Further research is required to determine if long-term static feeding of PB diets can meet and maintain AA and other nutrient targets in dogs.

POST-GASTRIC BYPASS HYPERAMMONEMIC ENCEPHALOPATHY: A RARE AND POTENTIALLY FATAL CAUSE OF ENCEPHALOPATHY IN THE ICU

TOPIC: Critical Care TYPE: Fellow Case Reports INTRODUCTION: In the US the prevalence of obesity has risen to 39.8% and affected about 93.3 million of US adults in 2015. Morbidly obese patients are increasingly pursuing bariatric surgery. While the benefits of bariatric surgery are numerous, it is important to recognize and understand the short term and long term postoperative complications. Hyperammonemic syndrome is an uncommon but severe complication of gastric bypass surgery. CASE PRESENTATION: A 43 year old woman with history of non-alcoholic fatty liver disease and history of prior gastric sleeve which was followed by Roux-en-Y Gastric Bypass (RYGB) surgery was admitted for shortness of breath and altered mental status. On hospital day number 10 patient was transferred to ICU for altered mental status, worsening hypoxia, retroperitoneal bleed and septic shock. Patient was subsequently intubated, given transfusions, placed on vasopressors, and given broad-spectrum antibiotics. After 72 hours her overall shock resolved and her ventilator requirements were minimal, but she remained in a comatose state. Initial workup including CT head and brain MRI were unremarkable. Initial routine EEG was consistent with low voltage delta frequencies which could be seen in the setting of severe encephalopathy. Ammonia level had risen to 169 umol/L, which was significantly elevated from prior level of 81 umol/L on admission (Table 1). In addition to aggressive treatment with lactulose and rifaximin, multiple supplements were added, including: Zinc, multivitamin, and L-carnitine. Subsequent EEG on ICU revealed myoclonic status epilepticus for which antiepileptic drugs were given. Repeat MRI of the brain revealed increased diffusion-weighted signal intensity within the cortex and subcortical white matter of suggesting hypoxic and anoxic brain injury. DISCUSSION: Hyperammonemic syndrome related to bariatric surgery is characterized by hyperammonemia, elevated plasma glutamine, hypoalbuminemia, reactive hypoglycemia, nutritional deficiencies of essential amino acids, and low zinc levels, in the absence of overt liver fibrosis or evidence of significant hepatocellular injury (1). The mortality rate approaches 50% and it tends to occur at a high rate in women (1). Patients may present with irritability, vomiting, ataxia, mental retardation, lethargy, and eventually alteration in consciousness and coma (1). There are multiple genetic and nongenetic hypothesized mechanisms (Table 2). Treatment strategies for hyperammonemia include: lactulose, rifaximin, repletion of deficient amino acids, zinc, micro nutrients, and prevention of seizures and cerebral edema (2). CONCLUSIONS: It is important to consider hyperammonemia related to bypass as a potential etiology for neurologic changes in patients with prior bypass, especially in the ICU setting where multiple etiologies can cause encephalopathy and hyperammonemia. REFERENCE #1: Fenves, A. et al. "Hyperammonemic Syndrome After Roux-En-Y Gastric Bypass." Obesity Journal. Volume 23. Number 4. 2015. REFERENCE #2: Krishnan P, Ramdas P, Landsberg. "Bariatric Surgery causing hyperammonemia." Cureus 11 (7): e5098. DOI 10.7759/cureus.5098. July 8, 2019 DISCLOSURES: No relevant relationships by SOHAIB KHAN, source=Web Response No relevant relationships by Jessica Nash, source=Web Response No relevant relationships by Mehul Patel, source=Web Response No relevant relationships by Mena Tawfik, source=Web Response

Monomethyl branched-chain fatty acid mediates amino acid sensing upstream of mTORC1

Animals have developed various nutrient-sensing mechanisms for survival under fluctuating environmental conditions. Although extensive cell-culture-based analyses have identified diverse mediators of amino acid sensing upstream of mTOR, studies using animal models to examine intestine-initiated amino acid sensing mechanisms under specific physiological conditions are lacking. Here, we developed a Caenorhabditis elegans model to examine the impact of amino acid deficiency on development. We discovered a leucine-derived monomethyl branched-chain fatty acid and its downstream metabolite, glycosphingolipid, which critically mediates the overall amino acid sensing by intestinal and neuronal mTORC1, which in turn regulates postembryonic development at least partly by controlling protein translation and ribosomal biogenesis. Additional data suggest that a similar mechanism may operate in mammals. This study uncovers an amino-acid-sensing mechanism mediated by a lipid biosynthesis pathway.

Gut microbiome in people living with HIV is associated with impaired thiamine and folate syntheses

People living with HIV have a high incidence of cardiovascular and neurological diseases as comorbid disorders that are commonly linked to inflammation. While microbial translocation can augment inflammation during HIV infection, functional microbiome shifts that may increase pro-inflammatory responses have not been fully characterized. In addition, defining HIV-induced microbiome changes has been complicated by high variability among individuals. Here we conducted functional annotation of previously-published 16S ribosomal RNA gene sequences of 305 HIV positive and 249 negative individuals, with adjustment for geographic region, sex, sexual behavior, and age. Metagenome profiles were inferred from these individuals' 16S data. HIV infection was associated with impaired microbial vitamin B synthesis; around half of the gene families in thiamine and folate biosynthesis pathways were significantly less abundant in the HIV positive group than the negative control. These results are consistent with the high prevalence of thiamine and folate deficiencies in HIV infections. These HIV-induced microbiota shifts have the potential to influence cardiovascular and neurocognitive diseases, given the documented associations between B-vitamin deficiencies, inflammation, and these diseases. We also observed that most essential amino acid biosynthesis pathways were downregulated in the microbiome of HIV-infected individuals. Microbial vitamin B and amino acid synthesis pathways were not significantly recovered by antiretroviral treatment when we compared 262 ART positive and 184 ART negative individuals. Our meta-analysis provides a new outlook for understanding vitamin B and amino acid deficiencies in HIV patients, suggesting that interventions for reversing HIV-induced microbiome shifts may aid in lessening the burdens of HIV comorbidities.

In vitro digestion characteristics of cereal protein concentrates as assessed using a pepsin-pancreatin digestion model

An alkaline extraction method has been used in many studies to extract total protein from cereal samples. Wheat bran protein concentrate (WBPC), oat bran protein concentrate (OBPC), and barley protein concentrate (BPC) were prepared by alkaline extraction and isoelectric precipitation to study their functional and nutritional properties. The three protein concentrates were hydrolysed by an in vitro pepsin-pancreatin digestion model. Their digestibility (%) and degree of hydrolysis (DH%) were evaluated, and SDS-PAGE electrophoresis was used to illustrate the protein and peptides patterns. The change of the particle sizes and the release of the essential amino acids was followed during the digestion process. The in vitro digestibility of WBPC, OBPC and BPC was 87.4%, 96.1% and 76.9%, respectively. The DH% of protein concentrates were between 50 and 60%. The change of the particle size distribution values Dv(50) was assumed to be related to protein aggregations during the digestion. The protein fractions were identified and the degradation during the digestion and were analysed by SDS-PAGE; the gels of WBPC and OBPC digestion showed virtually complete degradation whereas the intensive bands of undigested protein were presented for BPC. The generation of the free amino acids and short chain peptides were significantly higher at the end of the intestinal digestion compared to the stages of before and after gastric digestion. Higher content of the deficient amino acids such as lysine and threonine were found comparing to the level of deficient amino acids in cereal grains but does not meet the daily recommended intake.

MEF2D-NR4A1-FAM134B2-mediated reticulophagy contributes to amino acid homeostasis

ABSTRACT We recently identified FAM134B2, which is an N-terminal truncated reticulophagy receptor highly induced by starvation such as fasting of mice and treatment of mammalian cells with a starvation medium that does not contain amino acids, glucose and growth factors. However, which starvation signal mediates the induction of FAM134B2 is still obscure. In this study, we found that amino acid deficiency (AAD) could mimic the starvation condition to induce FAM134B2 expression. Unexpectedly, EIF2AK4/GCN2-mediated integrated signal response (ISR) and MTOR (mechanistic target of rapamycin kinase) signals, which constitute two major signaling pathways that respond to AAD, did not contribute to AAD-induced FAM134B2 induction. mRNA-seq and siRNA screenings identified two ISR-independent transcription factors, MEF2D (myocyte enhancer factor 2D) and NR4A1 (nuclear receptor subfamily 4 group A member 1), involved in AAD-induced FAM134B2 expression. AAD induces MEF2D, resulting in the induction of NR4A1, which in turn induces FAM134B2-mediated reticulophagy to maintain intracellular amino acid levels. In conclusion, the MEF2D-NR4A1-FAM134B2 cascade is a critical signal in amino acid homeostasis. Abbreviations AAD: amino acid deficiency; APOC3: apolipoprotein C3; BACH1: BTB domain and CNC homolog 1; CEBP: CCAAT enhancer binding protein; DDIT3/CHOP: DNA damage inducible transcript 3; EBSS: Earle’s Balanced Salt Solution; EIF2AK4/GCN2: eukaryotic translation initiation factor 2 alpha kinase 4; ER: endoplasmic reticulum; HisOH: histidinol; ISR: integrated stress response; MAP1LC3/LC3: microtubule associated protein 1 light chain 3; MEF2D: myocyte enhancer factor 2D; MTOR: mechanistic target of rapamycin kinase; NR4A1: nuclear receptor subfamily 4 group A member 1; RETREG1/FAM134B: reticulophagy regulator 1; RTN2: reticulon 2, TF: transcription factor; TFEB: transcription factor EB; ZBTB10: zinc finger and BTB domain containing 10

Hepatic Manifestations of Urea Cycle Disorders.

Content available: Author Interview and Audio Recording.

Animal source foods, rich in essential amino acids, are important for linear growth and development of young children in low- and middle-income countries.

Growth faltering under 5 years of age is unacceptably high worldwide, and even more children, while not stunted, fail to reach their growth potential. The time between conception and 2 years of age is critical for development. The period from 6 to 23 months, when complementary foods are introduced, coincides with a time when growth faltering and delayed neurocognitive developments are most common. Fortunately, this is also the period when diet exercises its greatest influence. Growing up in an adverse environment, with a deficient diet, as typically seen in low‐ and middle‐income countries (LMICs), hampers growth and development of children and prevents them from realising their full developmental and economic future potential. Sufficient nutrient availability and utilisation are paramount to a child's growth and development trajectory, especially in the period after breastfeeding. This review highlights the importance of essential amino acids (EAAs) in early life for linear growth and, likely, neurocognitive development. The paper further discusses signalling through mammalian target of rapamycin complex 1 (mTORC1) as one of the main amino acid (AA)‐sensing hubs and the master regulator of both growth and neurocognitive development. Children in LMICs, despite consuming sufficient total protein, do not meet their EAA requirements due to poor diet diversity and low‐quality dietary protein. AA deficiencies in early life can cause reductions in linear growth and cognition. Ensuring AA adequacy in diets, particularly through inclusion of nutrient‐dense animal source foods from 6 to 23 months, is strongly encouraged in LMICs in order to compensate for less than optimal growth during complementary feeding.