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3-hydroxyisovalerylcarnitine

Known as: 3-HIVA-carnitine 
 
National Institutes of Health

Papers overview

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2013
2013
Background:Children and adolescents with overt type 1 diabetes (T1D) have been found to show an altered carnitine profile. This… Expand
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2013
2013
BACKGROUND Biotin plays an important role as a covalently bound coenzyme for carboxylases. Carnitine is essential in β-oxidation… Expand
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2012
2012
Isolated 3-Methylcrotonyl-CoA carboxylase deficiency (MCC deficiency) is an organic aciduria presenting with a highly variable… Expand
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2011
2011
BACKGROUND The use of tandem mass spectrometry (MS/MS) for the analysis of amino acids and acylcarnitines from dried-blood spots… Expand
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2009
2009
A family with 3-methylcrotonyl-CoA carboxylase deficiency with different clinical features is described. A 15-month-old boy, who… Expand
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2009
2009
SummaryObjective: To report the clinical course and explore the gene mutation spectrum of four Chinese children with biotinidase… Expand
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2008
2008
Due to its increased concentration in blood, 3-hydroxyisovalerylcarnitine (C5OH-I) is an important indicator for the diagnosis of… Expand
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2006
2006
New technology enables expansion of newborn screening (NBS) of inborn errors aimed to prevent adverse outcome. In conditions with… Expand
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1995
1995
SummaryA new acylcarnitine was observed in the plasma and urine of a patient with isolated 3-methylcrotonyl-CoA carboxylase… Expand
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1995
1995
SummaryGenetic deficiency of 3-methylcrotonyl-CoA carboxylase (3-MCC) is a rare inborn error of leucine metabolism producing an… Expand
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