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Sucrase-isomaltase

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https://www.readbyqxmd.com/read/27872184/functional-variants-in-the-sucrase-isomaltase-gene-associate-with-increased-risk-of-irritable-bowel-syndrome
#1
Maria Henström, Lena Diekmann, Ferdinando Bonfiglio, Fatemeh Hadizadeh, Eva-Maria Kuech, Maren von Köckritz-Blickwede, Louise B Thingholm, Tenghao Zheng, Ghazaleh Assadi, Claudia Dierks, Martin Heine, Ute Philipp, Ottmar Distl, Mary E Money, Meriem Belheouane, Femke-Anouska Heinsen, Joseph Rafter, Gerardo Nardone, Rosario Cuomo, Paolo Usai-Satta, Francesca Galeazzi, Matteo Neri, Susanna Walter, Magnus Simrén, Pontus Karling, Bodil Ohlsson, Peter T Schmidt, Greger Lindberg, Aldona Dlugosz, Lars Agreus, Anna Andreasson, Emeran Mayer, John F Baines, Lars Engstrand, Piero Portincasa, Massimo Bellini, Vincenzo Stanghellini, Giovanni Barbara, Lin Chang, Michael Camilleri, Andre Franke, Hassan Y Naim, Mauro D'Amato
OBJECTIVE: IBS is a common gut disorder of uncertain pathogenesis. Among other factors, genetics and certain foods are proposed to contribute. Congenital sucrase-isomaltase deficiency (CSID) is a rare genetic form of disaccharide malabsorption characterised by diarrhoea, abdominal pain and bloating, which are features common to IBS. We tested sucrase-isomaltase (SI) gene variants for their potential relevance in IBS. DESIGN: We sequenced SI exons in seven familial cases, and screened four CSID mutations (p...
November 21, 2016: Gut
https://www.readbyqxmd.com/read/27865621/menaquinone-4-vitamin-k2-up-regulates-expression-of-human-intestinal-alkaline-phosphatase-in-caco-2-cells
#2
Seiko Noda, Asako Yamada, Rieko Tanabe, Kanae Nakaoka, Takayuki Hosoi, Masae Goseki-Sone
Alkaline phosphatase (ALP) hydrolyzes several monophosphate esters into inorganic acid and alcohol. In humans, 4 kinds of ALP isozymes have been identified: tissue-nonspecific ALP, intestinal ALP, placental ALP, and germ cell ALP. Intestinal ALP is expressed at a high concentration in the brush border membrane of intestinal epithelial cells and is known to be affected by several kinds of nutrients, such as lipids, but the physiological function of intestinal ALP has remained elusive. Vitamin K is an essential cofactor for the posttranslational carboxylation of glutamate residues into γ-carboxy glutamate...
October 14, 2016: Nutrition Research
https://www.readbyqxmd.com/read/27749612/congenital-sucrase-isomaltase-deficiency-a-novel-compound-heterozygous-mutation-causing-aberrant-protein-localization
#3
Yael Haberman, Ayelet Di Segni, Nurit Loberman-Nachum, Ortal Barel, Vered Kunik, Eran Eyal, Nitzan Kol, Goni Hout-Siloni, Brigitte Kochavi, Camila Avivi, Michael Schvimer, Gideon Rechavi, Yair Anikster, Iris Barshack, Batia Weiss
OBJECTIVES: Congenital chronic diarrhea (CCD) is a group of inherited enteropathies presenting in early life and requiring parenteral nutrition. In most cases, genetics may be the key for precise diagnosis. We present an infant girl with CCD that resolved after introduction of fructose-based formula but had no identified mutation in the SLC5A1 gene. Using whole exome sequencing (WES) we identified other mutations that better dictated dietary adjustments. METHODS: WES of the patient and her parents was performed...
October 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27173889/pigs-that-are-divergent-in-feed-efficiency-differ-in-intestinal-enzyme-and-nutrient-transporter-gene-expression-nutrient-digestibility-and-microbial-activity
#4
S Vigors, T Sweeney, C J O'Shea, A K Kelly, J V O'Doherty
Feed efficiency is an important trait in the future sustainability of pig production, however, the mechanisms involved are not fully elucidated. The objective of this study was to examine nutrient digestibility, organ weights, select bacterial populations, volatile fatty acids (VFA's), enzyme and intestinal nutrient transporter gene expression in a pig population divergent in feed efficiency. Male pigs (n=75; initial BW 22.4 kg SEM 2.03 kg) were fed a standard finishing diet for 43 days before slaughter to evaluate feed intake and growth for the purpose of calculating residual feed intake (RFI)...
May 13, 2016: Animal: An International Journal of Animal Bioscience
https://www.readbyqxmd.com/read/27166598/acetonic-extract-from-the-feijoa-sellowiana-berg-fruit-exerts-antioxidant-properties-and-modulates-disaccharidases-activities-in-human-intestinal-epithelial-cells
#5
Fabio Turco, Ilaria Palumbo, Paolo Andreozzi, Giovanni Sarnelli, Francesca De Ruberto, Giuseppe Esposito, Adriana Basile, Rosario Cuomo
Feijoa sellowiana fruit has been shown to possess various biological activities, such as anti-bacterial and anti-cancer properties, in a variety of cellular models, but its activity on human intestinal epithelial cells has never been tested. The purpose of this study was to investigate the effects of the acetonic extract of F. sellowiana fruits on the viability, membrane peroxidation, disaccharidases activities and proliferation of in vitro models of human intestinal epithelial cells. To obtain this goal, Caco-2 and HT-29 cells were exposed to the acetonic extract for 24 h...
August 2016: Phytotherapy Research: PTR
https://www.readbyqxmd.com/read/27162343/taste-cell-expressed-%C3%AE-glucosidase-enzymes-contribute-to-gustatory-responses-to-disaccharides
#6
Sunil K Sukumaran, Karen K Yee, Shusuke Iwata, Ramana Kotha, Roberto Quezada-Calvillo, Buford L Nichols, Sankar Mohan, B Mario Pinto, Noriatsu Shigemura, Yuzo Ninomiya, Robert F Margolskee
The primary sweet sensor in mammalian taste cells for sugars and noncaloric sweeteners is the heteromeric combination of type 1 taste receptors 2 and 3 (T1R2+T1R3, encoded by Tas1r2 and Tas1r3 genes). However, in the absence of T1R2+T1R3 (e.g., in Tas1r3 KO mice), animals still respond to sugars, arguing for the presence of T1R-independent detection mechanism(s). Our previous findings that several glucose transporters (GLUTs), sodium glucose cotransporter 1 (SGLT1), and the ATP-gated K(+) (KATP) metabolic sensor are preferentially expressed in the same taste cells with T1R3 provides a potential explanation for the T1R-independent detection of sugars: sweet-responsive taste cells that respond to sugars and sweeteners may contain a T1R-dependent (T1R2+T1R3) sweet-sensing pathway for detecting sugars and noncaloric sweeteners, as well as a T1R-independent (GLUTs, SGLT1, KATP) pathway for detecting monosaccharides...
May 24, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27161454/inhibition-of-mitogen-activated-protein-kinase-kinase-dna-methyltransferase-and-transforming-growth-factor-%C3%AE-promotes-differentiation-of-human-induced-pluripotent-stem-cells-into-enterocytes
#7
Nao Kodama, Takahiro Iwao, Tomoki Kabeya, Takashi Horikawa, Takuro Niwa, Yuki Kondo, Katsunori Nakamura, Tamihide Matsunaga
We previously reported that small-molecule compounds were effective in generating pharmacokinetically functional enterocytes from human induced pluripotent stem (iPS) cells. In this study, to determine whether the compounds promote the differentiation of human iPS cells into enterocytes, we investigated the effects of a combination of mitogen-activated protein kinase kinase (MEK), DNA methyltransferase (DNMT), and transforming growth factor (TGF)-β inhibitors on intestinal differentiation. Human iPS cells cultured on feeder cells were differentiated into endodermal cells by activin A...
June 2016: Drug Metabolism and Pharmacokinetics
https://www.readbyqxmd.com/read/26966071/modeling-of-drug-mediated-cyp3a4-induction-by-using-human-ips-cell-derived-enterocyte-like-cells
#8
Ryosuke Negoro, Kazuo Takayama, Yasuhito Nagamoto, Fuminori Sakurai, Masashi Tachibana, Hiroyuki Mizuguchi
Many drugs have potential to induce the expression of drug-metabolizing enzymes, particularly cytochrome P450 3A4 (CYP3A4), in small intestinal enterocytes. Therefore, a model that can accurately evaluate drug-mediated CYP3A4 induction is urgently needed. In this study, we overlaid Matrigel on the human induced pluripotent stem cells-derived enterocyte-like cells (hiPS-ELCs) to generate the mature hiPS-ELCs that could be applied to drug-mediated CYP3A4 induction test. By overlaying Matrigel in the maturation process of enterocyte-like cells, the gene expression levels of intestinal markers (VILLIN, sucrase-isomaltase, intestine-specific homeobox, caudal type homeobox 2, and intestinal fatty acid-binding protein) were enhanced suggesting that the enterocyte-like cells were maturated by Matrigel overlay...
April 15, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/26879013/chromosomal-and-genetic-analysis-of-a-human-lung-adenocarcinoma-cell-line-om
#9
Yong-Wu Li, Lin Bai, Lyu-Xia Dai, Xu He, Xian-Ping Zhou
BACKGROUND: Lung cancer has become the leading cause of death in many regions. Carcinogenesis is caused by the stepwise accumulation of genetic and chromosomal changes. The aim of this study was to investigate the chromosome and gene alterations in the human lung adenocarcinoma cell line OM. METHODS: We used Giemsa banding and multiplex fluorescence in situ hybridization focusing on the human lung adenocarcinoma cell line OM to analyze its chromosome alterations...
February 20, 2016: Chinese Medical Journal
https://www.readbyqxmd.com/read/26857124/the-clinical-consequences-of-sucrase-isomaltase-deficiency
#10
Stanley A Cohen
Primary sucrase-isomaltase deficiency, originally thought to be a homozygous recessive disorder, has been found to have numerous genetic variants that alone or in combination (compound heterozygosity) express varying degrees of clinical illness, most commonly causing chronic diarrhea, abdominal pain, and bloating. These symptoms are also present with secondary sucrase-isomaltase deficiency. Recent investigations are providing evidence that sucrase-isomaltase deficiency is more prevalent and of greater clinical significance than previously suspected...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/26830109/milk-glucosidase-activity-enables-suckled-pup-starch-digestion
#11
B L Nichols, M Diaz-Sotomayor, S E Avery, S K Chacko, D L Hadsell, S S Baker, B R Hamaker, L K Yan, H M Lin, R Quezada-Calvillo
UNLABELLED: ᅟ: Starch requires six enzymes for digestion to free glucose: two amylases (salivary and pancreatic) and four mucosal maltase activities; sucrase-isomaltase and maltase-glucoamylase. All are deficient in suckling rodents. OBJECTIVE: The objective of this study is to test (13)C-starch digestion before weaning by measuring enrichment of blood (13)C-glucose in maltase-glucoamylase-null and wild-type mice. METHODS: Maltase-glucoamylase gene was ablated at the N-terminal...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/26812950/the-multiple-roles-of-sucrase-isomaltase-in-the-intestinal-physiology
#12
Birthe Gericke, Mahdi Amiri, Hassan Y Naim
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...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/26553929/cargo-selective-apical-exocytosis-in-epithelial-cells-is-conducted-by-myo5b-slp4a-vamp7-and-syntaxin-3
#13
Georg F Vogel, Katharina M C Klee, Andreas R Janecke, Thomas Müller, Michael W Hess, Lukas A Huber
Mutations in the motor protein Myosin Vb (Myo5B) or the soluble NSF attachment protein receptor Syntaxin 3 (Stx3) disturb epithelial polarity and cause microvillus inclusion disease (MVID), a lethal hereditary enteropathy affecting neonates. To understand the molecular mechanism of Myo5B and Stx3 interplay, we used genome editing to introduce a defined Myo5B patient mutation in a human epithelial cell line. Our results demonstrate a selective role of Myo5B and Stx3 for apical cargo exocytosis in polarized epithelial cells...
November 9, 2015: Journal of Cell Biology
https://www.readbyqxmd.com/read/26163121/congenital-sucrase-isomaltase-deficiency-diagnostic-challenges-and-response-to-enzyme-replacement-therapy
#14
J W L Puntis, V Zamvar
Congenital sucrase-isomaltase (SI) deficiency is a rare genetic condition characterised by a deficiency in the brush-border SI enzyme, resulting in an inability to metabolise sucrose and starches. Six cases of congenital SI deficiency treated with Sucraid (sacrosidase, a yeast-derived enzyme that facilitates sucrose digestion) are described. Typical presenting symptoms were watery diarrhoea, abdominal pain and bloating, sometimes noticeably worse after ingestion of fruit. Diagnosis is challenging since conventional hydrogen breath testing after an oral sucrose load is impractical in young children, and many laboratories no longer look for maldigested sucrose using faecal sugar chromatography...
September 2015: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/26129821/histone-deacetylase-inhibition-impairs-normal-intestinal-cell-proliferation-and-promotes-specific-gene-expression
#15
Alireza Roostaee, Amel Guezguez, Marco Beauséjour, Aline Simoneau, Pierre H Vachon, Emile Levy, Jean-François Beaulieu
Mechanisms that maintain proliferation and delay cell differentiation in the intestinal crypt are not yet fully understood. We have previously shown the implication of histone methylation in the regulation of enterocytic differentiation. In this study, we investigated the role of histone deacetylation as an important epigenetic mechanism that controls proliferation and differentiation of intestinal cells using the histone deacetylase inhibitor suberanilohydroxamic acid (SAHA) on the proliferation and differentiation of human and mouse intestinal cells...
November 2015: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/26122626/the-pathobiochemistry-of-gastrointestinal-symptoms-in-a-patient-with-niemann-pick-type-c-disease
#16
Mahdi Amiri, Eva-Maria Kuech, Hadeel Shammas, Gabi Wetzel, Hassan Y Naim
The molecular basis of gastrointestinal intolerances in a severe case of Niemann-Pick type C disease was analyzed in an intestinal biopsy specimen. The enzyme activities of intestinal sucrase-isomaltase and maltase-glucoamylase are reduced in the patient, while that of lactase is comparable to the control. The association of SI with lipid rafts is reduced in the patient's biopsy as a consequence of altered composition of membrane microdomains. As association with lipid rafts influences the intracellular transport and the enzyme activities of sucrase-isomaltase and maltase-glucoamylase, these data explain reduced carbohydrate digestion in the intestinal lumen and delineate the effect of deficient cholesterol and sphingolipid homeostasis in development of gastrointestinal symptoms in NPC patients...
June 30, 2015: JIMD Reports
https://www.readbyqxmd.com/read/26122561/differential-transcriptional-responses-underlie-dietary-induction-of-intestinal-carbohydrase-activities-in-house-sparrow-nestlings
#17
C Gatica-Sosa, P Brzęk, J G Chediack, F D Cid, W H Karasov, E Caviedes-Vidal
Many species show diet-induced flexibility of activity of intestinal enzymes; however, molecular and genetic mechanisms responsible for such modulation are less known, particularly in altricial birds. The goal of our study was to test whether a diet-induced increase in activity of intestinal maltase and sucrase in house sparrow nestlings is matched with an increase in maltase-glucoamylase (MG) and sucrase-isomaltase (SI) complex mRNAs respectively. Both enzyme activities were significantly higher in mid-intestine of nestlings fed a medium-starch (MS) diet compared to those fed a starch-free (SF) diet...
April 2016: Journal of Animal Physiology and Animal Nutrition
https://www.readbyqxmd.com/read/26122164/lgr5-positive-stem-cells-sorted-from-small-intestines-of-diabetic-mice-differentiate-into-higher-proportion-of-absorptive-cells-and-paneth-cells-in-vitro
#18
Xian-Yang Zhong, Tao Yu, Wa Zhong, Jie-Yao Li, Zhong-Sheng Xia, Yu-Hong Yuan, Zhong Yu, Qi-Kui Chen
Intestinal epithelial stem cells (IESCs) can differentiate into all types of intestinal epithelial cells (IECs) and Leucine-rich repeat-containing G protein-coupled receptor 5 (Lgr5) is a marker for IESC. Previous studies reported enhanced proliferation of IECs in diabetic mice. In this study, the in vitro differentiation of Lgr5 positive IESCs sorted from diabetic mice was further investigated. The diabetic mouse model was induced by streptozotocin (STZ), and crypt IECs were isolated from small intestines...
August 2015: Development, Growth & Differentiation
https://www.readbyqxmd.com/read/26084276/a-double-blind-randomized-placebo-controlled-trial-studying-the-effects-of-saccharomyces-boulardii-on-the-gastrointestinal-tolerability-safety-and-pharmacokinetics-of-miglustat
#19
RANDOMIZED CONTROLLED TRIAL
Tatiana Remenova, Olivier Morand, Dominick Amato, Harbajan Chadha-Boreham, Scott Tsurutani, Thorsten Marquardt
BACKGROUND: Gastrointestinal (GI) disturbances such as diarrhea and flatulence are the most frequent adverse effects associated with miglustat therapy in type 1 Gaucher disease (GD1) and Niemann-Pick disease type C (NP-C), and the most common recorded reason for stopping treatment during clinical trials and in clinical practice settings. Miglustat-related GI disturbances are thought to arise from the inhibition of intestinal disaccharidases, mainly sucrase isomaltase. We report the effects of a co-administered dietary probiotic, S...
2015: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/25843107/design-and-synthesis-of-labystegines-hybrid-iminosugars-from-lab-and-calystegine-as-inhibitors-of-intestinal-%C3%AE-glucosidases-binding-conformation-and-interaction-for-ntsi
#20
Atsushi Kato, Zhao-Lan Zhang, Hong-Yao Wang, Yue-Mei Jia, Chu-Yi Yu, Kyoko Kinami, Yuki Hirokami, Yutaro Tsuji, Isao Adachi, Robert J Nash, George W J Fleet, Jun Koseki, Izumi Nakagome, Shuichi Hirono
This paper identifies the required configuration and orientation of α-glucosidase inhibitors, miglitol, α-1-C-butyl-DNJ, and α-1-C-butyl-LAB for binding to ntSI (isomaltase). Molecular dynamics (MD) calculations suggested that the flexibility around the keyhole of ntSI is lower than that of ctSI (sucrase). Furthermore, a molecular-docking study revealed that a specific binding orientation with a CH-π interaction (Trp370 and Phe648) is a requirement for achieving a strong affinity with ntSI. On the basis of these results, a new class of nortropane-type iminosugars, labystegines, hybrid iminosugars of LAB and calystegine, have been designed and synthesized efficiently from sugar-derived cyclic nitrones with intramolecular 1,3-dipolar cycloaddition or samarium iodide catalyzed reductive coupling reaction as the key step...
May 1, 2015: Journal of Organic Chemistry
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