3-hydroxyisovalerylcarnitine

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

Topic mentions per year

Topic mentions per year

1993-2016
012319932016

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2016
2016
We report here a case of maternal 3-methylcrotonyl-coenzyme A carboxylase (3-MCC) deficiency in a Korean woman. Her 2 infants had… (More)
  • table 1
  • figure 1
  • figure 2
Is this relevant?
2014
2014
3-methylcrotonyl-coenzyme A carboxylase (3MCC) deficiency is an autosomal recessive disorder in which leucine catabolism is… (More)
  • figure 1
Is this relevant?
2013
2013
BACKGROUND/PURPOSE In mice, biotin deficiency is one of the most potent clefting factors. Increased 3-hydroxyisovalerylcarnitine… (More)
Is this relevant?
2011
2011
BACKGROUND The use of tandem mass spectrometry (MS/MS) for the analysis of amino acids and acylcarnitines from dried-blood spots… (More)
Is this relevant?
2010
2010
We report a positive newborn screen for 3-hydroxyisovalerylcarnitine (C(5)OH) with an absence of 3-methylcrotonyl-coenzyme A… (More)
Is this relevant?
2009
2009
Objective: To report the clinical course and explore the gene mutation spectrum of four Chinese children with biotinidase… (More)
  • table 1
  • table 2
  • figure 1
  • figure 2
Is this relevant?
2008
2008
Due to its increased concentration in blood, 3-hydroxyisovalerylcarnitine (C5OH-I) is an important indicator for the diagnosis of… (More)
Is this relevant?
1995
1995
A new acylcarnitine was observed in the plasma and urine of a patient with isolated 3-methylcrotonyl-CoA carboxylase deficiency… (More)
  • figure 2
  • figure 3
  • figure 4
Is this relevant?
1995
1995
Genetic deficiency of 3-methylcrotonyl-CoA carboxylase (3-MCC) is a rare inborn error of leucine metabolism producing an organic… (More)
  • figure 1
Is this relevant?
1994
1994
Since biotin-deficient (BD) rats are a good animal model for human multiple carboxylase deficiency and have low plasma free… (More)
  • figure 1
  • figure 2
  • figure 3
  • figure 4
Is this relevant?