Congenital Sucrase‐Isomaltase Deficiency

Naim, Hassan Y.; Heisenberg, Martin; Zimmer, Klaus–Peter

doi:10.1097/01.mpg.0000421402.57633.4b

Cited by 39 publications

(44 citation statements)

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“…For N-terminal MGAM (NtMGAM) and C-terminal MGAM (CtMGAM) with 40% sequence identity, their substrate specificities are different due to the latter having both higher maltase and glucoamylase activities. 28 The importance of MGAM and SI to carbohydrate digestion is further shown by the disease of congenital sucrase-isomaltase deficiency 29 in which only MGAM is present as α-glucosidase. 25 Maltotriose, maltotetrose, and maltopentose all display a fairly strong substrate "brake" effect on MGAM activity 26 through the CtMGAM's "glucoamylase" subunit.…”

Section: Coordination Of Carbohydrate Property and Enzyme Activitymentioning

confidence: 99%

Gut feedback mechanisms and food intake: a physiological approach to slow carbohydrate bioavailability

et al. 2015

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Glycemic carbohydrates in foods are an important macronutrient providing the biological fuel of glucose for a variety of physiological processes. A classification of glycemic carbohydrates into rapidly digestible carbohydrate (RDC) and slowly digestible carbohydrate (SDC) has been used to specify their nutritional quality related to glucose homeostasis that is essential to normal functioning of the brain and critical to life. Although there have been many studies and reviews on slowly digestible starch (SDS) and SDC, the mechanisms of their slow digestion and absorption were mostly investigated from the material side without considering the physiological processes of their in vivo digestion, absorption, and most importantly interactions with other food components and the gastrointestinal tract. In this article, the physiological processes modulating the bioavailability of carbohydrates, specifically the rate and extent of their digestion and absorption as well as the related locations, in a whole food context, will be discussed by focusing on the activities of the gastrointestinal tract including glycolytic enzymes and glucose release, sugar sensing, gut hormones, and neurohormonal negative feedback mechanisms. It is hoped that a deep understanding of these physiological processes will facilitate the development of innovative dietary approaches to achieve desired carbohydrate or glucose bioavailability for improved health.

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Section: Coordination Of Carbohydrate Property and Enzyme Activitymentioning

confidence: 99%

Gut feedback mechanisms and food intake: a physiological approach to slow carbohydrate bioavailability

et al. 2015

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“…This study's outcome is higher than the frequency of known sucrase mutations, and our findings suggest that the sucrose intolerance may be acquired or that epigenetic factors may be in play. This present study was not designed to investigate the cause of sucrase insufficiency, which could be genetic [51, 52], acquired due to inflammation, cachexia, and anticancer therapy [53] or specifically related to the fructose component or the GLUT-5 transporter that might be involved [54]. …”

Section: Discussionmentioning

confidence: 99%

Use of the Biphasic¹³C-Sucrose/Glucose Breath Test to Assess Sucrose Maldigestion in Adults with Functional Bowel Disorders

Opekun

Balesh

Shelby

2016

BioMed Research International

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Sucrase insufficiency has been observed in children with of functional bowel disorders (FBD) and symptoms of dietary carbohydrate intolerance may be indistinguishable from those of FBD. A two-phase 13C-sucrose/13C-glucose breath test (13C-S/GBT) was used to assess sucrase activity because disaccharidase assays are seldom performed in adults. When 13C-sucrose is hydrolyzed to liberate monosaccharides, oxidation to 13CO2 is a proportional indicator of sucrase activity. Subsequently, 13C-glucose oxidation rate was determined after a secondary substrate ingestion (superdose) to adjust for individual habitus effects (Phase II). 13CO2 enrichment recovery ratio from 13C-sucrose and secondary 13C-glucose loads reflect the individualized sucrase activity [Coefficient of Glucose Oxidation for Sucrose (CGO-S)]. To determine if sucrase insufficiency could be a factor in FBD, 13C-S/GBT was validated using subjects with known sucrase gene mutation status by comparing 13CO2-breath enrichment with plasma 13C-glucose enrichment. 13C-S/GBT was used to assess sucrose digestion in FBD patients and asymptomatic controls. 13CO2-breath enrichment correlated with the appearance of 13C-sucrose-derived glucose in plasma (r 2 = 0.80). Mean, control group CGO-S-enrichment outcomes were 1.01 at 60′, 0.92 at 75′, and 0.96 at mean 60′–75′ with normal CGO-S defined as >0.85 (95% C.I.). In contrast, FBD patients demonstrated lower CGO-S values of 0.77 at 60′, 0.77 at 75′, and 0.76 at mean 60′–75′ (Chi Square: 6.55; p < 0.01), which points to sucrose maldigestion as a cause of FBD.

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“…[ 67,69 ] The two domain activities have been referred to as maltase Ib and maltase Ia, respectively. [ 63,70,72 ]…”

Section: Related Diseases/disordersmentioning

confidence: 99%

“…The incidence of primary sucrase‐isomaltase deficiency disease is typically 1/500 to 1/2000 (of live births), although in some populations the incidence is higher. [ 53,72–74 ]…”

Section: Related Diseases/disordersmentioning

confidence: 99%

“…Treatment (of chronic diarrhea, abdominal pain, and bloating) is achieved by restricting sucrose and starch in the diet. [ 53,72–75 ] However, this can be quite challenging as starch provides a large proportion of dietary calories. [ 65 ] A secondary (transient) form of the disease exists [ 74,76 ] with the same symptoms as the primary form.…”

Section: Related Diseases/disordersmentioning

confidence: 99%

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Lactose, Maltose, and Sucrose in Health and Disease

Qi¹,

Tester²

2020

Molecular Nutrition Food Res

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Scope: This review represents a focus on the structure and properties of the common nutritional disaccharides (lactose, maltose, and sucrose) in health and disease. The aim is to provide a comprehensive reference source related to the role of disaccharides in human nutrition. Methods and results: Key reference sources are searched, including Web of Science, PubMed, Science Direct, and Wiley Online, and key reference works are selected to support the factual basis of the text where interpretations and relevance of the works are discussed in the review. There are key nutritional health benefits of receiving dietary energy in the form of sugars, but equally life-threatening issues exist associated with constant/excess consumption. These issues are discussed together with genetic disorders, which impact upon health associated with consumption of the disaccharides (e.g., specific disaccharide intolerance due to deficiency of relevant digestive enzymes). Conclusions: As the three common dietary disaccharides (lactose, maltose, and sucrose) are consumed on a very regular basis in the human diet, it is critical to understand insofar as possible their role in health and disease. This review provides an insight into the structure and properties of these molecules in health and disease.

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Congenital Sucrase‐Isomaltase Deficiency

Abstract: The Heart and Stroke Foundation of Canada (NA-6305) provided an operating grant (to D.R.R.) and the Canadian Institutes for Health Research (FRN79400) provided an operating grant (to D.R.R. and B.M.P.

Cited by 39 publications

References 81 publications

Gut feedback mechanisms and food intake: a physiological approach to slow carbohydrate bioavailability

Gut feedback mechanisms and food intake: a physiological approach to slow carbohydrate bioavailability

Use of the Biphasic¹³C-Sucrose/Glucose Breath Test to Assess Sucrose Maldigestion in Adults with Functional Bowel Disorders

Lactose, Maltose, and Sucrose in Health and Disease

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Congenital Sucrase‐Isomaltase Deficiency

Abstract: The Heart and Stroke Foundation of Canada (NA-6305) provided an operating grant (to D.R.R.) and the Canadian Institutes for Health Research (FRN79400) provided an operating grant (to D.R.R. and B.M.P.

Cited by 39 publications

References 81 publications

Gut feedback mechanisms and food intake: a physiological approach to slow carbohydrate bioavailability

Gut feedback mechanisms and food intake: a physiological approach to slow carbohydrate bioavailability

Use of the Biphasic13C-Sucrose/Glucose Breath Test to Assess Sucrose Maldigestion in Adults with Functional Bowel Disorders

Lactose, Maltose, and Sucrose in Health and Disease

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Product

Resources

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Use of the Biphasic¹³C-Sucrose/Glucose Breath Test to Assess Sucrose Maldigestion in Adults with Functional Bowel Disorders