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3-hydroxyisovalerylcarnitine

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

Papers overview

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2017
2017
Importance Phosphodiesterase-5 inhibition with sildenafil compared with a placebo had no effect on the exercise capacity or… Expand
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2013
2013
Biotin plays an important role as a covalently bound coenzyme for carboxylases. Carnitine is essential in β‐oxidation to… Expand
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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2012
2012
Isolated 3-Methylcrotonyl-CoA carboxylase deficiency (MCC deficiency) is an organic aciduria presenting with a highly variable… Expand
2011
2011
BACKGROUND The use of tandem mass spectrometry (MS/MS) for the analysis of amino acids and acylcarnitines from dried-blood spots… Expand
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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2008
2008
Due to its increased concentration in blood, 3-hydroxyisovalerylcarnitine (C5OH-I) is an important indicator for the diagnosis of… Expand
2006
2006
SummaryA new acylcarnitine was observed in the plasma and urine of a patient with isolated 3-methylcrotonyl-CoA carboxylase… Expand
Highly Cited
2006
Highly Cited
2006
New technology enables expansion of newborn screening (NBS) of inborn errors aimed to prevent adverse outcome. In conditions with… Expand
2004
2004
SummaryGenetic deficiency of 3-methylcrotonyl-CoA carboxylase (3-MCC) is a rare inborn error of leucine metabolism producing an… Expand