Milk glucosidase activity enables suckled pup starch digestion
Conclusions
(1) 13C-α-limit dextrins were rapidly digested to 13C-glucose in 13-day mice independent of maltase-glucoamylase genotype or mucosal maltase activity. (2) This experiment demonstrates that a soluble maltase activity is secreted in mouse mother’s milk which enables suckling pup starch digestion well before brush border enzyme development. (3) This experiment with 13C-α-limit dextrins needs to be repeated in human breast fed infants. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - February 1, 2016 Category: Cytology Source Type: research
Towards understanding microvillus inclusion disease
Abstract
Microvillus inclusion disease (MVID) is characterised by onset of intractable life-threatening watery diarrhoea during infancy. Transmission electron microscopy demonstrates shortening or absence of apical microvilli, pathognomonic microvillus inclusions in mature enterocytes and subapical accumulation of periodic acid-Schiff-positive granules or vesicles confirming diagnosis. Mutations in MYO5B have been found to cause MVID. In two patients with MVID, whole-exome sequencing of DNA revealed homozygous truncating mutations in STX3. Mutations in these genes disrupt trafficking between apical carg...
Source: Molecular and Cellular Pediatrics - January 29, 2016 Category: Cytology Source Type: research
The multiple roles of sucrase-isomaltase in the intestinal physiology
Abstract
Osmotic diarrhea and abdominal pain in humans are oftentimes associated with carbohydrate malabsorption in the small intestine due to loss of function of microvillar disaccharidases. Disaccharidases are crucial for the digestion and the subsequent absorption of carbohydrates. This review focuses on sucrase-isomaltase as the most abundant intestinal disaccharidase and the primary or induced pathological conditions that affect its physiological function. Congenital defects are primary factors which directly influence the transport and function of sucrase-isomaltase in a healthy epithelium. Based ...
Source: Molecular and Cellular Pediatrics - January 26, 2016 Category: Cytology Source Type: research
The role of hypoxia in intestinal inflammation
Abstract
Inflammatory bowel disease (IBD) is a chronic relapsing inflammatory disease of the intestine. IBD is a multifactorial disorder, and IBD-associated genes are critical in innate immune response, inflammatory response, autophagy, and epithelial barrier integrity. Moreover, epithelial oxygen tension plays a critical role in intestinal inflammation and resolution in IBD. The intestines have a dynamic and rapid fluctuation in cellular oxygen tension, which is dysregulated in IBD. Intestinal epithelial cells have a steep oxygen gradient where the tips of the villi are hypoxic and the oxygenation incr...
Source: Molecular and Cellular Pediatrics - January 26, 2016 Category: Cytology Source Type: research
Expression and function of histamine and its receptors in atopic dermatitis
Conclusions
With this review, we provide an overview on histamine and the expression of its four receptors on skin resident and nonresident cells. Furthermore, we summarize recent studies which suggest anti-histamine therapy to efficiently combat pruritus and inflammation in atopic dermatitis and discuss possible approaches to incorporate these findings into more effective treatment strategies for atopic dermatitis in childhood. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - December 21, 2015 Category: Cytology Source Type: research
Pluripotent stem cells for disease modeling and drug screening: new perspectives for treatment of cystic fibrosis?
Abstract
Despite continuous improvements in treating clinical symptoms and the identification of single compounds that effectively rescue some rare mutations in the cystic fibrosis transmembrane conductance regulator (CFTR), associated lung and liver pathologies remain largely untreatable and no real breakthrough is visible for the majority of patients suffering from cystic fibrosis (CF).
Novel compounds have to be identified and tailored in combination to specific CFTR mutations, to different tissues, or even to the individual patient. Immortalized cell lines overexpressing mutant C...
Source: Molecular and Cellular Pediatrics - December 14, 2015 Category: Cytology Source Type: research
Post stroke hemi-dystonia in children: a neglected area of research
Conclusions
Dystonia after CAIS is a common yet insufficiently understood and poorly studied clinical challenge. Studies to improve our understanding of the underlying pathophysiology and consequently the development of instruments for early prediction as well as targeted treatment of dystonia should become a high priority in collaborative childhood stroke research. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - December 11, 2015 Category: Cytology Source Type: research
Airway reactivity and sphingolipids—implications for childhood asthma
Abstract
Asthma is a clinically heterogeneous disorder, whose onset and progression results from a complex interplay between genetic susceptibility, allergens, and viral triggers. Sphingolipids and altered sphingolipid metabolism have emerged as potential key contributors to the pathogenesis of asthma. Orosomucoid-like 3 gene (ORMDL3) and the asthma susceptibility locus 17q21 have been strongly and reproducibly linked to childhood asthma, but how this gene is functionally linked to asthma is incompletely understood. ORMDL proteins play an integral role in sphingolipid homeostasis and synthesis, and asth...
Source: Molecular and Cellular Pediatrics - December 4, 2015 Category: Cytology Source Type: research
GM-CSF and the role of myeloid regulatory cells in the pathogenesis and treatment of Crohn’s disease
Conclusions
This review article summarizes recent developments in the immunology of mucosal diseases and describes new aspects of the role of myeloid regulatory cells in IBD and the function of GM-CSF in maintaining the intestinal immune homeostasis in Crohn’s disease (CD). This review article highlights the exploration of stimulating in addition to suppressive therapies for patients with IBD and underpins that myeloid regulatory cells might become a promising novel cell-based therapeutic option. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - December 1, 2015 Category: Cytology Source Type: research
Modified mRNA as a new therapeutic option for pediatric respiratory diseases and hemoglobinopathies
Conclusions
This mini review summarizes the recent advances in modified mRNA-based therapy and its potential therapeutic effect in treating major pediatric diseases. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - November 20, 2015 Category: Cytology Source Type: research
Stent implantation into the tracheo-bronchial system in rabbits: histopathologic sequelae in bare metal vs. drug-eluting stents
Conclusions
After stent implantation in the tracheo-bronchial system of rabbits, we found tissue reactions comparable to those seen after stent implantation into the vascular system. There was no difference between coated and uncoated stents with regard to quality and quantity of tissue proliferation. We found, however, a significantly different inflammatory reaction with a more pronounced foreign-body reaction in sirolimus-coated stents. In our small series, drug-eluting stents did not exhibit any benefit over bare metal stents in an experimental setting. (Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - November 5, 2015 Category: Cytology Source Type: research
Lessons learned from metabolomics in cystic fibrosis
Abstract
Cystic fibrosis is a mono-genetic multi-system disease; however, respiratory manifestations cause the main morbidity and mortality where chronic bacterial infections lead to bronchiectasis and ultimately respiratory failure. Metabolomics allows a relatively complete snapshot of metabolic processes in a sample using different mass spectrometry methods. Sample types used for discovery of biomarkers or pathomechanisms in cystic fibrosis (CF) have included blood, respiratory secretions, and exhaled breath to date. Metabolomics has shown distinction of CF vs. non-CF for matrices of blood, exhaled br...
Source: Molecular and Cellular Pediatrics - October 20, 2015 Category: Cytology Source Type: research
A translational approach to dystroglycanopathies: a frequent type of muscular dystrophy
(Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - July 1, 2015 Category: Cytology Source Type: research
Mutations in the NKX2.1 and the PAX8 genes in a boy with thyroid dysgenesis, respiratory and neurological disorders
(Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - July 1, 2015 Category: Cytology Source Type: research
TMEM18 is a regulator of adipogenesis and involved in PPARG signalling in vivo
(Source: Molecular and Cellular Pediatrics)
Source: Molecular and Cellular Pediatrics - July 1, 2015 Category: Cytology Source Type: research
