RESUMO
Abdominal pain has been associated with disaccharidase deficiencies. While relationships with individual symptoms have been assessed, relationships between disaccharidase deficiencies and symptom complexes or inflammation have not been evaluated in this group. The primary aims of the current study were to assess relationships between disaccharidase deficiency and symptoms or symptom complexes and duodenal inflammation, respectively. Patients with abdominal pain who underwent endoscopy with evaluation of disaccharidase activity levels were identified. After excluding all patients with inflammatory bowel disease, celiac disease, H. pylori, or gross endoscopic lesions, patients were evaluated for disaccharidase deficiency frequency. Disaccharidase were compared between patients with and without histologic duodenitis. Lastly, relationships between individual gastrointestinal symptoms or symptom complexes were evaluated. Lactase deficiency was found in 34.3% of patients and disaccharidase pan-deficiency in 7.6%. No individual symptoms or symptom complexes predicted disaccharidase deficiency. While duodenitis was not associated with disaccharidase deficiency, it was only present in 5.9% of patients. Disaccharidase deficiency, particularly lactase deficiency, is common in youth with abdominal pain and multiple deficiencies are not uncommon. Disaccharidase deficiency cannot be predicted by symptoms in this population. Further studies are needed to assess the clinical significance of disaccharidase deficiency.
Assuntos
Dor Abdominal/metabolismo , Dissacaridases/deficiência , Duodenite/metabolismo , Inflamação/metabolismo , Adolescente , Criança , Feminino , Humanos , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: Tests for stool reducing sugars and stool pH are ordered for children with osmotic diarrhea to screen for carbohydrate malabsorption. METHODS: We compared the results of the two screening tests, stool reducing sugars and stool pH, with a more definitive result from an intestinal tissue disaccharidase activity assay ordered for pediatric patients (<18 years old). Overall, 159 patients had results for tissue disaccharidase and stool reducing sugars, but only 115 had additional results of stool pH. Forty-six of the 159 patients had mild, moderate, or severe disaccharidase deficiencies. The sensitivity and specificity of the screening tests were calculated for individual disaccharidase deficiencies. In addition, trends of abnormal tissue disaccharidase, stool reducing sugars, and stool pH results were examined in different age groups. RESULTS: The sensitivities for stool reducing sugars and stool pH were 9% to 28% and specificities were 74% to 81% for individual disaccharidase deficiencies. Infants (0 years of age) had the highest percentage of abnormal results across all three tests; however, the positive predicative values were 54% and 50% for stool reducing sugars and stool pH, respectively. CONCLUSIONS: The screening tests, stool reducing sugars and stool pH, had low sensitivity compared with results of measured tissue disaccharidase activity in pediatric patients. Infants had the highest percentage of abnormal results for all three tests, but the screening tests still performed poorly in that age group. This study suggests that stool reducing sugars and stool pH should not be used as screening tests for carbohydrate malabsorption due to disaccharidase deficiencies in pediatric patients.
Assuntos
Testes Diagnósticos de Rotina , Dissacaridases/deficiência , Fezes/química , Concentração de Íons de Hidrogênio , Síndromes de Malabsorção/diagnóstico , Síndromes de Malabsorção/etiologia , Açúcares/análise , Criança , Pré-Escolar , Testes Diagnósticos de Rotina/métodos , Testes Diagnósticos de Rotina/normas , Diarreia/diagnóstico , Diarreia/etiologia , Feminino , Humanos , Lactente , Masculino , Sensibilidade e EspecificidadeRESUMO
PURPOSE OF REVIEW: Disaccharidase testing, as applied to the evaluation of gastrointestinal disturbances is available but it is not routinely considered in the diagnostic work-up. The purpose of this review was to determine if disaccharidase testing is clinically useful and to consider how the results could alter patient management. RECENT FINDINGS: Indicate that carbohydrate maldigestion could contribute functional bowel disorders and negatively impact the fecal microbiome. Diagnostic techniques include enzyme activity assays performed on random endoscopically obtained small intestinal biopsies, immunohistochemistry, stable isotope tracer and nonenriched substrate load breath testing, and genetic testing for mutations. More than 40 sucrase--isomaltase gene variants coding for defective or reduced enzymatic activity have been reported and deficiency conditions are more common than previously thought. SUMMARY: The rationale for disaccharidase activity testing relates to a need to fully assess unexplained recurrent abdominal discomfort and associated symptoms. All disaccharidases share the same basic mechanism of mucosal expression and deficiency has far reaching consequences. Testing for disaccharidase expression appears to have an important role in symptom evaluation, but there are accuracy and logistical issues that should be considered. It is likely that specific recommendations for patient management, dietary modification, and enzyme supplementation would come from better testing methods.
Assuntos
Dissacaridases/análise , Gastroenteropatias/diagnóstico , Dissacaridases/deficiência , Dissacaridases/metabolismo , Fermentação , Gastroenteropatias/metabolismo , Gastroenteropatias/fisiopatologia , Microbioma Gastrointestinal/fisiologia , Humanos , Síndromes de Malabsorção/diagnóstico , Síndromes de Malabsorção/metabolismo , Síndromes de Malabsorção/fisiopatologiaRESUMO
OBJECTIVES: Disaccharidase (DS) activity in duodenal biopsy specimens is the gold standard for diagnosing DS deficiency. We investigated strategies to reduce the need for DS testing and whether clinical or histopathologic factors predict DS deficiency. METHODS: A retrospective chart review analyzed 1,678 DS results in children, biopsy indication(s), and duodenal histopathology. RESULTS: One or more DSs were abnormal in 42.8%. Sufficient lactase predicted sucrase, palatinase, and maltase sufficiency (negative predictive value 97.7%). Three patients had sucrase-isomaltase deficiency (0.2%). DS deficiency was more common in biopsy specimens for positive celiac serology (78.0%). Villous blunting, intraepithelial lymphocytosis, and active inflammation predicted DS deficiency; a combination of any two had an 81.4% positive predictive value. CONCLUSIONS: Utilization could be reduced by only testing cases with normal duodenal histopathology and ongoing clinical suspicion for DS deficiency after reviewing pathology. In cases with suspected celiac disease and/or mucosal injury, DS deficiency is common and likely secondary, limiting test utility.
Assuntos
Dissacaridases/deficiência , Gastroenteropatias/diagnóstico , Gastroenteropatias/etiologia , Adolescente , Biópsia , Criança , Pré-Escolar , Duodeno/patologia , Feminino , Gastroenteropatias/patologia , Humanos , Lactente , Lactase/deficiência , Masculino , Estudos Retrospectivos , Sacarase/deficiência , alfa-Glucosidases/deficiênciaRESUMO
There is overwhelming evidence that functional gastrointestinal disorders (FGIDs) are associated with specific mechanisms that constitute important targets for personalized treatment. There are specific mechanisms in patients presenting with functional upper gastrointestinal symptoms (UGI Sx). Among patients with UGI Sx, approximately equal proportions (25%) of patients have delayed gastric emptying (GE), reduced gastric accommodation (GA), both impaired GE and GA, or neither, presumably due to increased gastric or duodenal sensitivity. Treatments targeted to the underlying pathophysiology utilize prokinetics, gastric relaxants, or central neuromodulators. Similarly, specific mechanisms in patients presenting with functional lower gastrointestinal symptoms, especially with diarrhea or constipation, are recognized, including at least 30% of patients with functional constipation pelvic floor dyssynergia and 5% has colonic inertia (with neural or interstitial cells of Cajal loss in myenteric plexus); 25% of patients with diarrhea-predominant irritable bowel syndrome (IBSD) has evidence of bile acid diarrhea; and, depending on ethnicity, a varying proportion of patients has disaccharidase deficiency, and less often sucrose-isomaltase deficiency. Among patients with predominant pain or bloating, the role of fermentable oligosaccharides, disaccharides, monosaccharides and polyols should be considered. Personalization is applied through pharmacogenomics related to drug pharmacokinetics, specifically the role of CYP2D6, 2C19 and 3A4 in the use of drugs for treatment of patients with FGIDs. Single mutations or multiple genetic variants are relatively rare, with limited impact to date on the understanding or treatment of FGIDs. The role of mucosal gene expression in FGIDs, particularly in IBS-D, is the subject of ongoing research. In summary, the time for personalization of FGIDs, based on deep phenotyping, is here; pharmacogenomics is relevant in the use of central neuromodulators. There is still unclear impact of the role of genetics in the management of FGIDs.
Assuntos
Fármacos Gastrointestinais/farmacologia , Gastroenteropatias/tratamento farmacológico , Neurotransmissores/farmacologia , Variantes Farmacogenômicos , Medicina de Precisão/métodos , Citocromo P-450 CYP2C19/genética , Citocromo P-450 CYP2D6/genética , Citocromo P-450 CYP3A/genética , Dissacaridases/deficiência , Dissacaridases/genética , Fármacos Gastrointestinais/uso terapêutico , Gastroenteropatias/diagnóstico , Gastroenteropatias/etiologia , Gastroenteropatias/genética , Motilidade Gastrointestinal/efeitos dos fármacos , Motilidade Gastrointestinal/genética , Humanos , Neurotransmissores/uso terapêutico , Diafragma da Pelve/fisiopatologia , Resultado do TratamentoRESUMO
Disaccharidase deficiencies are reportedly underdiagnosed in pediatric populations. Though typically thought to cause diarrheal disease, they can also be a cause of abdominal pain and dyspepsia, and patients diagnosed with these functional disorders may actually have associated enzyme deficiencies. While the effects of lactose deficiency have been widely studied, sucrase, maltase, and isomaltase are less frequently considered when approaching a patient with an apparent functional abdominal pain disorder. This review seeks to provide an up-to-date narrative on the current scientific literature on the possible role of sucrase, maltase, and isomaltase deficiency in pediatric functional gastrointestinal disorders.
Assuntos
Dor Abdominal , Erros Inatos do Metabolismo dos Carboidratos , Dissacaridases/deficiência , Adolescente , Criança , Pré-Escolar , Dispepsia , Doença de Depósito de Glicogênio Tipo II , Humanos , Lactente , Síndrome do Intestino Irritável , Complexo Sacarase-Isomaltase/deficiênciaRESUMO
OBJECTIVES: The epidemiology and clinical significance of disaccharidase deficiencies have not been thoroughly characterized. Recent work suggests at least genetic sucrase-isomaltase deficiency is more prevalent than previously believed. Because lactase deficiency (LD) is well described, the present study focuses on the clinical characteristics of children with disaccharidase deficiencies determined by esophagogastroduodenoscopy. METHODS: Endoscopic records were reviewed from patients undergoing esophagogastroduodenoscopies with biopsies assayed for disaccharidase activity performed by 13 pediatric gastroenterologists during 5 years (2010-2014). Presenting symptoms, clinical and histological diagnosis, treatment, disaccharidase results, and demographic variables were obtained from medical and endoscopic records of those with maltase and sucrase deficiency (SD). RESULTS: Among 963 patients undergoing intestinal disaccharidase testing, 73 (7.6%) had SD on biopsy (enzyme activity <25âµmolâ·âminâ·âg). Thirty-four (34/73; 47%) had normal duodenal histology and are the focus of this report. Four patients had SD without LD. Pan-disaccharidase deficiency was observed in 24 patients when maltase and palatinase assays were obtained (nâ=â646), and 11 had SDâ+âLD when just those 2 enzymes were analyzed (nâ=â317). Those with SD without LD were younger 4.6â±â6.1 versus 14.1â±â3.6 years and uniformly presented with diarrhea. Patients with pan-disaccharidase deficiency or SDâ+âLD primarily reported abdominal pain (33/35; 94%), diarrhea (16/35; 46%), nausea (14/35; 40%); and poor weight gain/weight loss (10/35; 29%); constipation, flatulence, and bloating were also noted. Maltase deficiency is less common (8/963; 0.8%), presenting with similar symptoms. CONCLUSIONS: Genetic sucrase-isomaltase deficiency often occurs together with lactase or pan-disaccharide deficiency. Disaccharidase deficiency should be considered a potential cause of abdominal pain and/or diarrhea in children and adolescents.
Assuntos
Dissacaridases/deficiência , Duodeno/enzimologia , Síndromes de Malabsorção/diagnóstico , Adolescente , Criança , Pré-Escolar , Dissacaridases/análise , Endoscopia do Sistema Digestório/métodos , Feminino , Humanos , Lactente , Síndromes de Malabsorção/epidemiologia , Masculino , Prevalência , Estudos RetrospectivosRESUMO
BACKGROUND: A subset of children with functional gastrointestinal disorders (FGIDs), which includes functional dyspepsia, may have duodenal disaccharidase deficiencies. OBJECTIVES: To determine the frequency, demographics, and clinical characteristics associated with duodenal disaccharidase deficiencies in children with functional dyspepsia. METHODS: Children ages 4 to 18 years undergoing esophagogastroduodenoscopy (EGD) evaluation for dyspepsia were enrolled in either a retrospective (study 1) or prospective (study 2) evaluation. Those with histologic abnormalities were excluded. Duodenal biopsies were obtained for disaccharidase enzyme analysis. In the retrospective study, both demographic and clinical characteristics were obtained via chart review. In the prospective study, parents completed the Rome II Questionnaire on Gastrointestinal Symptoms before the EGD. RESULTS: One hundred and twenty-nine children (nâ=â101, study 1; nâ=â28, study 2) were included. Mean age was 11.2â±â3.8 (SD) years in study 1 and 10.6â±â3.2 years in study 2. Forty-eight (47.5%) of subjects in study 1 and 13 (46.4%) of subjects in study 2 had at least 1 disaccharidase deficiency identified. All of those with a disaccharidase deficiency in both studies had lactase deficiency with 8 (7.9%) and 5 (17.9%) of those in studies 1 and 2, respectively, having an additional disaccharidase deficiency. The second most common disaccharidase deficiency pattern was that of pan-disaccharidase deficiency (PDD) in both studies. In study 1 (where both race and ethnicity were captured), self-identified Hispanic (vs non-Hispanic, Pâ<â0.05) and non-white (vs white, Pâ<â0.01) children were more likely to have lactase deficiency. Age, sex, and type of gastrointestinal symptom were not associated with presence or absence of a disaccharidase deficiency. CONCLUSIONS: Approximately half of children with functional dyspepsia undergoing EGD were identified as having a disaccharidase deficiency (predominantly lactase deficiency). Race/ethnicity may be associated with the likelihood of identifying a disaccharidase deficiency. Other clinical characteristics were not able to distinguish those with versus without a disaccharidase deficiency.
Assuntos
Dissacaridases/deficiência , Duodeno/enzimologia , Dispepsia/etiologia , Mucosa Intestinal/enzimologia , Síndromes de Malabsorção/epidemiologia , Adolescente , Criança , Pré-Escolar , Duodeno/patologia , Endoscopia do Sistema Digestório , Feminino , Humanos , Mucosa Intestinal/patologia , Síndromes de Malabsorção/complicações , Síndromes de Malabsorção/diagnóstico , Masculino , Estudos Prospectivos , Estudos RetrospectivosRESUMO
AIM: To elucidate the role of intestinal carbohydrases (glucoamylase, maltase, sucrose, and lactase) in the etiology and pathogenesis of functional bowel diseases (FBD). SUBJECTS AND METHODS: 74 patients (36 men and 38 women) aged 18 to 50 years with FBD were examined. According to Rome IV criteria (2016), there was diarrhea-predominant irritable bowel syndrome (IBS) in 21 patients, functional diarrhea (FD) in 33, constipation-predominant IBS in 6, functional constipation (FC) in 4, and mixed IBS in 10. The activity of carbohydrases in the small intestine mucosa (SIM) was investigated by the Dahlquist method modified by Trinder in the duodenal biopsy specimens obtained during esophagogastroduodenoscopy. RESULTS: Lactase deficiency was identified in 87.8% of the patients; maltase deficiency in 48.6%; sucrose deficiency in 51.3%; and glucoamylase deficiency in 85.1%. The activity of all the investigated enzymes was reduced in 23 (31.1%) patients with FBD; deficiency of 1-3 carbohydrases was found in 47 (63.5%). Normal enzymatic activity was established in 4 (5.4%) patients. CONCLUSION: In the majority of patients with FBD, the intestinal symptoms are caused by the decreased activity of SIM carbohydrases. Therefore, disaccharidase deficiency associated with an established damaging agent (nonsteroidal anti-inflammatory drugs, antibiotics, acute intestinal infections, etc.) should be considered to be a more precise diagnosis.
Assuntos
Dissacaridases , Síndrome do Intestino Irritável , Síndromes de Malabsorção , Adolescente , Adulto , Constipação Intestinal , Diarreia , Dissacaridases/deficiência , Feminino , Humanos , Síndrome do Intestino Irritável/complicações , Síndrome do Intestino Irritável/enzimologia , Síndromes de Malabsorção/enzimologia , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
OBJECTIVES: Carbohydrate intolerance or malabsorption has been suggested as a cause of chronic abdominal pain (CAP) in a subset of patients. We aimed to evaluate disaccharidase deficiencies in children with functional CAP and to correlate deficiencies with clinical features. METHOD: Patients presenting to the gastroenterology clinic at Children's Hospital of Wisconsin with abdominal pain prospectively completed a detailed demographic, history, and symptom questionnaire. The CAP cohort included those with at least 1 month of symptoms. Data on disaccharidase activity and histology of endoscopic biopsies were collected retrospectively. Only patients with normal histology were included in the study. The association between groups with low disaccharidases and clinical features was examined. RESULTS: A total of 203 pediatric patients with CAP were included. The mean (SD) age was 11.5 (3.1) years, and 32.5% were male. The percentages of abnormally low disaccharidase levels using the standard laboratory cutoffs were lactase, 37%; sucrase, 21%; glucoamylase, 25%; and palatinase, 8%. Thirty-nine percent of the patients with low lactase also had low sucrase, and 67% of the patients with low sucrase had low lactase. There was no significant difference in the activities of any of the disaccharidases or sucrase/lactase ratio in relation to age. Also, no association was found between stool consistency, stool frequency, or location of pain and low disaccharidase activity. CONCLUSIONS: A large proportion of patients with CAP have deficiencies in disaccharidases. Bowel frequency, vomiting, or location of pain was no different between groups, suggesting that these clinical features cannot be used to predict disaccharidase deficiencies.
Assuntos
Dor Abdominal/sangue , Erros Inatos do Metabolismo dos Carboidratos/sangue , Dissacaridases/sangue , Dissacaridases/deficiência , Síndromes de Malabsorção/sangue , Dor Abdominal/etiologia , Adolescente , Erros Inatos do Metabolismo dos Carboidratos/complicações , Criança , Doença Crônica , Dissacaridases/administração & dosagem , Dissacaridases/metabolismo , Feminino , Glucana 1,4-alfa-Glucosidase/metabolismo , Humanos , Lactase/metabolismo , Síndromes de Malabsorção/complicações , Masculino , Estudos Prospectivos , Estudos Retrospectivos , Sacarase/metabolismo , WisconsinRESUMO
Intolerance to foods which contain lactose can cause a range of intestinal and systemic symptoms. These symptoms are caused by Lactase deficiency which is encoded by a single gene (LCT) of ≈ 50 kb located on chromosome 2q21. In some food items, lactose has been missed because of "hidden" lactose due to inadequately labeled, confusing diagnosis of lactose intolerance based on dietary restriction of dairy foods. Two polymorphisms, C/T13910 and G/A22018, linked to hypolactasia, correlate with breath hydrogen and symptoms after lactose. The key in the management of lactose intolerance is the dietary removal of lactose. Patients diagnosed as lactose intolerant must be advised of "risk" foods, inadequately labeled, including processed meats, bread, cake mixes, soft drinks, and lagers. This review highlights the types, symptoms and management of lactose intolerance and also highlights differences from milk allergy which closely mimics the symptoms of lactose intolerance.
Assuntos
Intolerância à Lactose , Criança , Dissacaridases/deficiência , Humanos , Intolerância à Lactose/diagnóstico , Intolerância à Lactose/terapiaRESUMO
Disaccharide intolerance is the inability to digest certain carbohydrates due to a lack of one or more intestinal disaccharidases (e.g., lactase, maltase, isomaltase and sucrase). Symptoms include diarrhea, abdominal distention and flatulence. Management of the disorder by external enzymes supplementation has not yet been attempted. We report that the medicinal plant Tinospora cordifolia contains substantial amounts of all disaccharidases required for intestinal digestion of carbohydrates. The plant is also a rich source of saccharifying amylase. We recovered (units/100 g fresh stem) amylase: 49,000+500, maltase: 400+50, isomaltase: 130+50, sucrase: 4500+500, acid lactase: 350+30, cellobiase: 35+10 and trehalase: 40+10 by buffer extraction of the blended stem. Crude enzymes in the forms of stem powder, lyophilized aqueous extract and ethanol precipitated protein were found to be stable. Disaccharidases were optimally active at 50 (0) C in the pH range of 4-5. Lactase was an acid lactase similar to the type linked with human lactose intolerance. Enzymes were catalytically stable in the pH range of 2-7 and temperature range of up to 40 (0) C. T. cordifolia enzyme was non-toxic up to a dose of 200 mg protein/kg body weight.
Assuntos
Dissacaridases/administração & dosagem , Síndromes de Malabsorção/tratamento farmacológico , Fitoterapia , Tinospora/enzimologia , Animais , Dissacaridases/deficiência , Dissacaridases/isolamento & purificação , Extratos Vegetais/química , Extratos Vegetais/uso terapêutico , Plantas Medicinais/enzimologia , Ratos , Ratos Wistar , Testes de Toxicidade AgudaAssuntos
Dissacaridases/deficiência , Dissacarídeos/metabolismo , Mucosa Intestinal/enzimologia , Intestino Delgado/enzimologia , Complexo Sacarase-Isomaltase/deficiência , Adolescente , Adulto , Biópsia , Erros Inatos do Metabolismo dos Carboidratos/epidemiologia , Erros Inatos do Metabolismo dos Carboidratos/metabolismo , Criança , Pré-Escolar , Dissacaridases/metabolismo , Humanos , Lactente , Mucosa Intestinal/patologia , Intestino Delgado/patologia , Prevalência , Complexo Sacarase-Isomaltase/metabolismo , Adulto JovemAssuntos
Erros Inatos do Metabolismo dos Carboidratos/dietoterapia , Carboidratos da Dieta/metabolismo , Dissacaridases/deficiência , Mucosa Intestinal/enzimologia , Fenótipo , Adolescente , Adulto , Erros Inatos do Metabolismo dos Carboidratos/metabolismo , Criança , Pré-Escolar , Dissacaridases/metabolismo , Humanos , Lactente , Grupos de Autoajuda , Complexo Sacarase-Isomaltase/deficiência , Complexo Sacarase-Isomaltase/metabolismo , Adulto JovemRESUMO
BACKGROUND AND AIMS: The "gold standard" for the diagnosis of celiac disease (CD) is the small intestinal biopsy. A significant number of biopsies are inadequate for interpretation. Furthermore, the labeling of a biopsy as a Marsh I or II is somewhat subjective and may vary with the experience of the pathologist. Our hypothesis is that patients with intact villi undergoing biopsies frequently have associated disaccharidase deficiencies (DSD). METHODS: We reviewed 220 charts of pediatric patients with CD and selected those with a duodenal biopsy Marsh score of I/II. The disaccharidase (DS) levels of these patients were compared with a randomly selected, age-matched control group. DSD is defined as levels below the lower limits of normal. RESULTS: Lactase (mean lactase = 18.8 in the control group vs. 4.2 in the diseased group, p = 0.004); sucrase (mean sucrase = 46.4 in the control group vs. 21.4 in the diseased group, p = 0.001); maltase (mean maltase = 138 in the control group vs. 52.5 in the diseased group, p = 0.001); palatinase (mean palatinase = 9.6 in the control group vs. 3.3 in the diseased group, p < 0.001). CONCLUSION: There is a profound deficiency of DS levels in pediatric patients with CD who have intact villi.
Assuntos
Doença Celíaca/enzimologia , Doença Celíaca/patologia , Dissacaridases/deficiência , Duodeno/enzimologia , Mucosa Intestinal/enzimologia , Biópsia , Estudos de Casos e Controles , Criança , Dissacaridases/análise , Duodeno/patologia , Feminino , Humanos , Mucosa Intestinal/patologia , Lactase/análise , Modelos Logísticos , Masculino , Microvilosidades/patologia , Valor Preditivo dos Testes , Sacarase/análise , alfa-Glucosidases/análiseRESUMO
The food-borne pathogen Listeria monocytogenes is a problem for food processors and consumers alike, as the organism is resistant to harsh environmental conditions and inimical barriers implemented to prevent the survival and/or growth of harmful bacteria. One mechanism by which listeriae mediate survival is through the accumulation of compatible solutes, such as proline, betaine and carnitine. In other bacteria, including Escherichia coli, the synthesis and accumulation of another compatible solute, trehalose, are known to aid in the survival of stressed cells. The objective of this research was to investigate trehalose metabolism in L. monocytogenes, where the sugar is thought to be transferred across the cytoplasmic membrane via a specific phosphoenolpyruvate phosphotransferase system and phosphorylation to trehalose-6-phosphate (T6P). The latter is subsequently broken down into glucose and glucose-6-phosphate by α,α-(1,1) phosphotrehalase, the putative product of the treA gene. Here we report on an isogenic treA mutant of L. monocytogenes 568 (568:ΔTreA) which, relative to the wild-type strain, displays increased tolerances to multiple stressors, including heat, high osmolarity, and desiccation. This is the first study to examine the putative trehalose operon in L. monocytogenes, and we demonstrate that lmo1254 (treA) in L. monocytogenes 568 indeed encodes a phosphotrehalase required for the hydrolysis of T6P. Disruption of the treA gene results in the accumulation of T6P which is subsequently dephosphorylated to trehalose in the cytosol, thereby contributing to the stress hardiness observed in the treA mutant. This study highlights the importance of compatible solutes for microbial survival in adverse environments.
Assuntos
Dissacaridases/metabolismo , Temperatura Alta , Listeria monocytogenes/fisiologia , Pressão Osmótica , Estresse Fisiológico , Trealose/metabolismo , Dessecação , Dissacaridases/deficiência , Dissacaridases/genética , Deleção de Genes , Listeria monocytogenes/efeitos dos fármacos , Listeria monocytogenes/metabolismo , Listeria monocytogenes/efeitos da radiação , Viabilidade Microbiana/efeitos dos fármacos , Viabilidade Microbiana/efeitos da radiação , Fosfatos Açúcares/metabolismo , Trealose/análogos & derivadosRESUMO
Sugars normally are absorbed in the small intestine. When carbohydrates are malabsorbed, the osmotic load produced by the high amount of low molecular weight sugars and partially digested starches in the small intestine can cause symptoms of intestinal distention, rapid peristalsis, and diarrhea. Colonic bacteria normally metabolize proximally malabsorbed dietary carbohydrate through fermentation to small fatty acids and gases (ie, hydrogen, methane, and carbon dioxide). When present in large amounts, the malabsorbed sugars and starches can be excreted in the stool. Sugar intolerance is the presence of abdominal symptoms related to the proximal or distal malabsorption of dietary carbohydrates. The symptoms consist of meal-related abdominal cramps and distention, increased flatulence, borborygmus, and diarrhea. Infants and young children with carbohydrate malabsorption show more intense symptoms than adults; the passage of undigested carbohydrates through the colon is more rapid and is associated with detectable carbohydrates in copious watery acid stools. Dehydration often follows feeding of the offending sugar. In this review we present the clinical and current molecular aspects of disaccharidase digestion.
Assuntos
Digestão/genética , Digestão/fisiologia , Dissacaridases/deficiência , Dissacarídeos/metabolismo , Síndromes de Malabsorção/genética , Síndromes de Malabsorção/metabolismo , Dissacaridases/genética , Humanos , Mutação/genéticaRESUMO
Disorders of dietary sugar assimilation occur more often among native people of the Arctic then in temperate climate inhabitants. It is hypothesized that the limited variety of natural exogenous sugars in the Arctic, and their low content in the traditional diets of native northerners in accordance with a "protein-lipid" type of metabolism weakened selection, favoring diversity of disaccharidase enzymes.