Browsing by Subject "DEFICIENCIES"

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  • Forsström, Saara; Jackson, Christopher B.; Carroll, Christopher J.; Kuronen, Mervi; Pirinen, Eija; Pradhan, Swagat; Marmyleva, Anastasiia; Auranen, Mari; Kleine, Iida-Marja; Khan, Nahid A.; Roivainen, Anne; Marjamäki, Paivi; Liljenbäck, Heidi; Wang, Liya; Battersby, Brendan J.; Richter, Uwe; Velagapudi, Vidya; Nikkanen, Joni; Euro, Liliya; Suomalainen, Anu (2019)
    Mitochondrial dysfunction elicits stress responses that safeguard cellular homeostasis against metabolic insults. Mitochondrial integrated stress response (ISRmt) is a major response to mitochondrial (mt)DNA expression stress (mtDNA maintenance, translation defects), but the knowledge of dynamics or interdependence of components is lacking. We report that in mitochondrial myopathy, ISRmt progresses in temporal stages and development from early to chronic and is regulated by autocrine and endocrine effects of FGF21, a metabolic hormone with pleiotropic effects. Initial disease signs induce transcriptional ISRmt (ATF5, mitochondria) one-carbon cycle, FGF21, and GDF15). The local progression to 2nd metabolic ISRmt stage (ATF3, ATF4, glucose uptake, serine biosynthesis, and transsulfuration) is FGF21 dependent. Mitochondria! unfolded protein response marks the 3rd ISRmt stage of failing tissue. Systemically, FGF21 drives weight loss and glucose preference, and modifies metabolism and respiratory chain deficiency in a specific hippocampal brain region. Our evidence indicates that FGF21 is a local and systemic messenger of mtDNA stress in mice and humans with mitochondrial disease.
  • Hukkinen, Maria; Merras-Salmio, Laura; Pakarinen, Mikko P. (2018)
    Treatment results of pediatric intestinal failure have improved markedly during the last decades. With improved survival the attention is turning to other essential outcomes including quality of life and neurodevelopment. So far, relatively few studies with limited number of patients and variable methodology have addressed these issues. Based on these studies using generic health related quality of life tools, children with intestinal failure demonstrate decreased physical health, while PN-dependence is also associated with compromised emotional functioning. Impairments of social functioning are frequently observed among older children and parents. Few recent studies on neurodevelopment imply significant impairments in motor and mental skills among children with intestinal failure despite small sample sizes and limited follow-up times. Development of a disease-specific survey designed for the pediatric intestinal failure population could better reveal the health issues with greatest impact on quality of life. Robust studies with appropriate methodology on neurodevelopment in pediatric intestinal failure with extended follow-up times are urgently needed. Quality of life and neurodevelopment requires greater attention from medical professionals managing children with intestinal failure. (C) 2018 Elsevier Inc. All rights reserved.
  • Tamene, Aynadis; Kariluoto, Susanna; Baye, Kaleab; Humblot, Christele (2019)
    Injera is an Ethiopian fermented flatbread preferably made from whole grain cereal (tef). Tef it is increasingly used to produce gluten-free pasta and bread, but the folate content of teff and products made from it remains unknown. Given that folate deficiencies lead to several health disorders, the aim of this study was to quantify folate in each of the three main steps of traditional processing of tef injera. Total folate contents of tef flour, fermented batter and injera were determined through microbiological assays using Lactobacillus rhamnosus (ATCC 7469). Folate content of tef flour was 8.7 mu g/100 g of dry matter content, which is in the same range as the richest cereals like oats. The increase in folate content due to fermentation was highly variable (60-148%). Cooking always led to folate losses, with a maximum of 52.8%. Altogether, injera processing increased folate retention between 38.0 and 121.8%. Folate content of injera was 14.3 mu g/100 g on fresh weight-basis. Tef injera can contribute up to 10% of the recommended nutrient intake of folate for children aged 1-3 and women of reproductive age. Although the folate content of teff is already high, future studies should focus on optimizing the folate content of injera.
  • Tanaka, Atsushi; Ide, Tomomi; Fujino, Takeo; Onitsuka, Ken; Ikeda, Masataka; Takehara, Takako; Hata, Yuko; Ylikallio, Emil; Tyynismaa, Henna; Suomalainen, Anu; Sunagawa, Kenji (2013)
  • Bationo, Fabrice; Humblot, Christele; Songre-Ouattara, Laurencia T.; Hama-Ba, Fatoumata; Le Merrer, Manon; Chapron, Morgane; Kariluoto, Susanna; Hemery, Youna M. (2020)
    Cereal-based fermented foods (CBFF) are frequently consumed by young children in West-Africa. Seven CBFF types (gelatinized doughs, porridges, dumplings, and fritters, made from corn, sorghum or pearl millet) were produced according to the commonly used methods in traditional production units in Burkina Faso. Their total folate content, analyzed using a microbiological method, was between 1.8 and 31.3 mu g/100 g fresh weight. Folate degradation occurred at several steps during the cereal grain processing into CBFF, for instance during debranning (-51%), soaking (-20% to -49%) and wet-milling (-39% to -59%). Conversely, fermentation increased the folate content of some CBFF (+ 9 to + 27%). Folate bioaccessibility, assessed using a static in vitro digestion model, ranged from 23% to 81%. The bioaccessible folate content was influenced by the total folate content, the food matrix structure, and folate stability. CBFF contribution to the reference folate intake for young children was at most 23%. This work generated new information on the influence of traditional processing on the vitamin B9 content of CBFF, and the first data on folate bioaccessibility in West African CBFF.