Source:http://linkedlifedata.com/resource/pubmed/id/11502802
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
8
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pubmed:dateCreated |
2001-8-14
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pubmed:abstractText |
Mutations of glutamate dehydrogenase cause the hyperinsulinism/hyperammonemia syndrome by desensitizing glutamate dehydrogenase to allosteric inhibition by GTP. Normal allosteric activation of glutamate dehydrogenase by leucine is thus uninhibited, leading us to propose that children with hyperinsulinism/hyperammonemia syndrome will have exaggerated acute insulin responses to leucine in the postabsorptive state. As hyperglycemia increases beta-cell GTP, we also postulated that high glucose concentrations would extinguish abnormal responsiveness to leucine in hyperinsulinism/hyperammonemia syndrome patients. After an overnight fast, seven hyperinsulinism/hyperammonemia syndrome patients (aged 9 months to 29 yr) had acute insulin responses to leucine performed using an iv bolus of L-leucine (15 mg/kg) administered over 1 min and plasma insulin measurements obtained at -10, -5, 0, 1, 3, and 5 min. The acute insulin response to leucine was defined as the mean increase in insulin from baseline at 1 and 3 min after an iv leucine bolus. The hyperinsulinism/hyperammonemia syndrome group had excessively increased insulin responses to leucine (mean +/- SEM, 73 +/- 21 microIU/ml) compared with the control children and adults (n = 17) who had no response to leucine (1.9 +/- 2.7 microU/ml; P < 0.05). Four hyperinsulinism/hyperammonemia syndrome patients then had acute insulin responses to leucine repeated at hyperglycemia (blood glucose, 150-180 mg/dl). High blood glucose suppressed their abnormal baseline acute insulin responses to leucine of 180, 98, 47, and 28 microU/ml to 73, 0, 6, and 19 microU/ml, respectively. This suppression suggests that protein-induced hypoglycemia in hyperinsulinism/hyperammonemia syndrome patients may be prevented by carbohydrate loading before protein consumption.
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pubmed:grant |
http://linkedlifedata.com/resource/pubmed/grant/F32 DK009985-02,
http://linkedlifedata.com/resource/pubmed/grant/M01-RR-00240,
http://linkedlifedata.com/resource/pubmed/grant/R01-53012,
http://linkedlifedata.com/resource/pubmed/grant/R01-DK-56268,
http://linkedlifedata.com/resource/pubmed/grant/T32-DK-07314
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
AIM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Ammonia,
http://linkedlifedata.com/resource/pubmed/chemical/Blood Glucose,
http://linkedlifedata.com/resource/pubmed/chemical/Diazoxide,
http://linkedlifedata.com/resource/pubmed/chemical/Glutamate Dehydrogenase,
http://linkedlifedata.com/resource/pubmed/chemical/Insulin,
http://linkedlifedata.com/resource/pubmed/chemical/Leucine
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pubmed:status |
MEDLINE
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pubmed:month |
Aug
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pubmed:issn |
0021-972X
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
86
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
3724-8
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pubmed:dateRevised |
2011-11-17
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pubmed:meshHeading |
pubmed-meshheading:11502802-Adolescent,
pubmed-meshheading:11502802-Adult,
pubmed-meshheading:11502802-Amino Acid Substitution,
pubmed-meshheading:11502802-Ammonia,
pubmed-meshheading:11502802-Blood Glucose,
pubmed-meshheading:11502802-Child,
pubmed-meshheading:11502802-Child, Preschool,
pubmed-meshheading:11502802-Diazoxide,
pubmed-meshheading:11502802-Female,
pubmed-meshheading:11502802-Glutamate Dehydrogenase,
pubmed-meshheading:11502802-Humans,
pubmed-meshheading:11502802-Hyperammonemia,
pubmed-meshheading:11502802-Hyperinsulinism,
pubmed-meshheading:11502802-Infant,
pubmed-meshheading:11502802-Insulin,
pubmed-meshheading:11502802-Leucine,
pubmed-meshheading:11502802-Male,
pubmed-meshheading:11502802-Point Mutation,
pubmed-meshheading:11502802-Syndrome
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pubmed:year |
2001
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pubmed:articleTitle |
Acute insulin responses to leucine in children with the hyperinsulinism/hyperammonemia syndrome.
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pubmed:affiliation |
Division of Endocrinology, The Children's Hospital of Philadelphia, and Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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