Abstract
Background: The effect of insulin on glucose, protein metabolism, circulating fatty acids (FFA), potassium (K+) and C-peptide concentrations were investigated in a 12-year-old girl with leprechaunism. The mutations do not affect the insulin-receptor binding affinity and insulin-stimulated auto-phosphorylation of the receptor. Methods: The subject was studied with a primed-continuous infusion of [6,6-2H2] glucose and [1-13C] leucine during a basal period followed by two steps of insulin infusion (1 and 10 mU/kg/min) of 2 h each, during which plasma glucose level decreased from 131 to 115 and then to 95 mg/dL. Results: Whole body glucose disposal was virtually unaffected by insulin, slightly decreasing from 21 μmol/kg/min in the basal period to 20 and to 19 μmol/kg/min during the two steps of insulin infusion, respectively. The endogenous leucine flux, an index of proteolysis, was completely insensitive to insulin, being 182, 189 and 180 μmol/kg/min, in the three periods, respectively. The FFA concentration (an indirect index of lipolysis) decreased from 1135 to 799 during step 1. During step 2 the FFA concentration rebounded to 917 μmol/L. The concentration of K+ decreased from 4.2 to 3.2 mmol/L and an infusion of 20 mEq/h of KCl was necessary to prevent further hypokalemia (final value 3.3 mmol/1). The C-peptide concentration declined from 1.85 to 0.97 and then to 0.29 pmol/mL. Conclusions: The dissociation of control exerted by insulin on K+ uptake and on β-cell secretion may rely on a differential expression and folding of the mutated receptors in the different insulin target tissues.
| Original language | English |
|---|---|
| Pages (from-to) | 205-210 |
| Number of pages | 6 |
| Journal | Diabetes/Metabolism Research and Reviews |
| Volume | 24 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Mar 2008 |
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Keywords
- Free fatty acid
- Glucose metabolism
- Insulin resistance
- Leprechaunism
- Potassium and C-peptide
- Protein metabolism
ASJC Scopus subject areas
- Endocrinology
- Biochemistry
- Endocrinology, Diabetes and Metabolism
Cite this
Insulin effect on serum potassium and auto-inhibition of insulin secretion is intact in a patient with leprechaunism despite severe impairment of substrates metabolism. / Luzi, Livio; Zoppini, Giacomo; Targher, Giovanni; Battezzati, Alberto; Muggeo, Michele; Bonora, Enzo.
In: Diabetes/Metabolism Research and Reviews, Vol. 24, No. 3, 03.2008, p. 205-210.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Insulin effect on serum potassium and auto-inhibition of insulin secretion is intact in a patient with leprechaunism despite severe impairment of substrates metabolism
AU - Luzi, Livio
AU - Zoppini, Giacomo
AU - Targher, Giovanni
AU - Battezzati, Alberto
AU - Muggeo, Michele
AU - Bonora, Enzo
PY - 2008/3
Y1 - 2008/3
N2 - Background: The effect of insulin on glucose, protein metabolism, circulating fatty acids (FFA), potassium (K+) and C-peptide concentrations were investigated in a 12-year-old girl with leprechaunism. The mutations do not affect the insulin-receptor binding affinity and insulin-stimulated auto-phosphorylation of the receptor. Methods: The subject was studied with a primed-continuous infusion of [6,6-2H2] glucose and [1-13C] leucine during a basal period followed by two steps of insulin infusion (1 and 10 mU/kg/min) of 2 h each, during which plasma glucose level decreased from 131 to 115 and then to 95 mg/dL. Results: Whole body glucose disposal was virtually unaffected by insulin, slightly decreasing from 21 μmol/kg/min in the basal period to 20 and to 19 μmol/kg/min during the two steps of insulin infusion, respectively. The endogenous leucine flux, an index of proteolysis, was completely insensitive to insulin, being 182, 189 and 180 μmol/kg/min, in the three periods, respectively. The FFA concentration (an indirect index of lipolysis) decreased from 1135 to 799 during step 1. During step 2 the FFA concentration rebounded to 917 μmol/L. The concentration of K+ decreased from 4.2 to 3.2 mmol/L and an infusion of 20 mEq/h of KCl was necessary to prevent further hypokalemia (final value 3.3 mmol/1). The C-peptide concentration declined from 1.85 to 0.97 and then to 0.29 pmol/mL. Conclusions: The dissociation of control exerted by insulin on K+ uptake and on β-cell secretion may rely on a differential expression and folding of the mutated receptors in the different insulin target tissues.
AB - Background: The effect of insulin on glucose, protein metabolism, circulating fatty acids (FFA), potassium (K+) and C-peptide concentrations were investigated in a 12-year-old girl with leprechaunism. The mutations do not affect the insulin-receptor binding affinity and insulin-stimulated auto-phosphorylation of the receptor. Methods: The subject was studied with a primed-continuous infusion of [6,6-2H2] glucose and [1-13C] leucine during a basal period followed by two steps of insulin infusion (1 and 10 mU/kg/min) of 2 h each, during which plasma glucose level decreased from 131 to 115 and then to 95 mg/dL. Results: Whole body glucose disposal was virtually unaffected by insulin, slightly decreasing from 21 μmol/kg/min in the basal period to 20 and to 19 μmol/kg/min during the two steps of insulin infusion, respectively. The endogenous leucine flux, an index of proteolysis, was completely insensitive to insulin, being 182, 189 and 180 μmol/kg/min, in the three periods, respectively. The FFA concentration (an indirect index of lipolysis) decreased from 1135 to 799 during step 1. During step 2 the FFA concentration rebounded to 917 μmol/L. The concentration of K+ decreased from 4.2 to 3.2 mmol/L and an infusion of 20 mEq/h of KCl was necessary to prevent further hypokalemia (final value 3.3 mmol/1). The C-peptide concentration declined from 1.85 to 0.97 and then to 0.29 pmol/mL. Conclusions: The dissociation of control exerted by insulin on K+ uptake and on β-cell secretion may rely on a differential expression and folding of the mutated receptors in the different insulin target tissues.
KW - Free fatty acid
KW - Glucose metabolism
KW - Insulin resistance
KW - Leprechaunism
KW - Potassium and C-peptide
KW - Protein metabolism
UR - http://www.scopus.com/inward/record.url?scp=40749102077&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=40749102077&partnerID=8YFLogxK
U2 - 10.1002/dmrr.771
DO - 10.1002/dmrr.771
M3 - Article
C2 - 17968975
AN - SCOPUS:40749102077
VL - 24
SP - 205
EP - 210
JO - Diabetes/Metabolism Research and Reviews
JF - Diabetes/Metabolism Research and Reviews
SN - 1520-7552
IS - 3
ER -