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Low Fasting Concent...
Low Fasting Concentrations of Glucagon in Patients with Very Long-Chain Acyl-CoA Dehydrogenase Deficiency
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- Stenlid, Rasmus (author)
- Uppsala universitet,Institutionen för medicinsk cellbiologi
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- Manell, Hannes (author)
- Uppsala universitet,Institutionen för kvinnors och barns hälsa
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- Seth, Rikard (author)
- Uppsala universitet,Institutionen för medicinsk cellbiologi
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- Cerenius, Sara Y. (author)
- Uppsala universitet,Institutionen för medicinsk cellbiologi
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- Chowdhury, Azazul Islam (author)
- Uppsala universitet,Institutionen för medicinsk cellbiologi
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- Roa Cortes, Camilla (author)
- Uppsala universitet,Institutionen för medicinsk cellbiologi
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- Nyqvist, Isabelle (author)
- Uppsala universitet,Institutionen för medicinsk cellbiologi
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- Lundqvist, Thomas (author)
- Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm SE-17177, Sweden.
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- Halldin, Maria (author)
- Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm SE-17177, Sweden.
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- Bergsten, Peter (author)
- Uppsala universitet,Institutionen för medicinsk cellbiologi,Uppsala Univ, Dept Med Cell Biol, Uppsala SE-75123, Sweden.
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(creator_code:org_t)
- MDPI AG, 2023
- 2023
- English.
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In: Metabolites. - : MDPI AG. - 2218-1989 .- 2218-1989. ; 13:7
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https://uu.diva-port... (primary) (Raw object)
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https://urn.kb.se/re...
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Abstract
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- (1) Background: Deficiencies of mitochondrial fatty acid oxidation (FAO) define a subgroup of inborn errors of metabolism, with medium-chain acyl-CoA dehydrogenase deficiency (MCAD) and very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) being two of the most common. Hypoketotic hypoglycemia is a feared clinical complication and the treatment focuses on avoiding hypoglycemia. In contrast, carnitine uptake deficiency (CUD) is treated as a mild disease without significant effects on FAO. Impaired FAO has experimentally been shown to impair glucagon secretion. Glucagon is an important glucose-mobilizing hormone. If and how glucagon is affected in patients with VLCAD or MCAD remains unknown. (2)Methods: A cross-sectional study was performed with plasma hormone concentrations quantified after four hours of fasting. Patients with VLCAD (n = 10), MCAD (n = 7) and CUD (n = 6) were included. (3)Results: The groups were similar in age, sex, weight, and height. The glucagon and insulin levels were significantly lower in the VLCAD group compared to the CUD group (p < 0.05, respectively). The patients with CUD had glucagon concentrations similar to the normative data. No significant differences were seen in GLP-1, glicentin, glucose, amino acids, or NEFAs. (4)Conclusions: Low fasting concentrations of glucagon are present in patients with VLCAD and cannot be explained by altered stimuli in plasma.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Endokrinologi och diabetes (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Endocrinology and Diabetes (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Cell- och molekylärbiologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Cell and Molecular Biology (hsv//eng)
Keyword
- glucagon
- insulin
- glucagon like peptide-1
- very long-chain acyl-CoA dehydrogenase deficiency
- medium-chain acyl-CoA dehydrogenase deficiency
- carnitine uptake deficiency
- inborn errors of metabolism
- pediatrics
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Stenlid, Rasmus
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Manell, Hannes
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Seth, Rikard
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Cerenius, Sara Y ...
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Chowdhury, Azazu ...
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Roa Cortes, Cami ...
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Nyqvist, Isabell ...
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Lundqvist, Thoma ...
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Halldin, Maria
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Bergsten, Peter
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Endocrinology an ...
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Basic Medicine
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and Cell and Molecul ...
- Articles in the publication
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Metabolites
- By the university
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Uppsala University
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Karolinska Institutet