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3-hydroxyisovalerylcarnitine

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

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2 relations

Broader (1)

Carnitine
analogs & derivatives

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2017
2017
The metabolic response in fish to mildly elevated water temperature relates to species-dependent muscular concentrations of imidazole compounds and free amino acids.
2017
2017
Sildenafil Treatment in Heart Failure With Preserved Ejection Fraction: Targeted Metabolomic Profiling in the RELAX Trial
Importance Phosphodiesterase-5 inhibition with sildenafil compared with a placebo had no effect on the exercise capacity or… 
2013
2013
Biotin and carnitine profiles in preterm infants in Japan
Biotin plays an important role as a covalently bound coenzyme for carboxylases. Carnitine is essential in β‐oxidation to… 
2013
2013
Children who develop type 1 diabetes early in life show low levels of carnitine and amino acids at birth: does this finding shed light on the etiopathogenesis of the disease?
Background:Children and adolescents with overt type 1 diabetes (T1D) have been found to show an altered carnitine profile. This… 
2012
2012
A single mutation in MCCC1 or MCCC2 as a potential cause of positive screening for 3-methylcrotonyl-CoA carboxylase deficiency.
2011
2011
Evaluation of valproate effects on acylcarnitine in epileptic children by LC–MS/MS
2009
2009
3-Methylcrotonyl-CoA Carboxylase Deficiency: Phenotypic Variability in a Family
A family with 3-methylcrotonyl-CoA carboxylase deficiency with different clinical features is described. A 15-month-old boy, who… 
2009
2009
Diagnosis, treatment, follow-up and gene mutation analysis in four Chinese children with biotinidase deficiency
SummaryObjective: To report the clinical course and explore the gene mutation spectrum of four Chinese children with biotinidase… 
1995
1995
3-Hydroxyisovalerylcarnitine in 3-methylcrotonyl-CoA carboxylase deficiency
SummaryA new acylcarnitine was observed in the plasma and urine of a patient with isolated 3-methylcrotonyl-CoA carboxylase… 
1995
1995
Glycine andl-carnitine therapy in 3-methylcrotonyl-CoA carboxylase deficiency
SummaryGenetic deficiency of 3-methylcrotonyl-CoA carboxylase (3-MCC) is a rare inborn error of leucine metabolism producing an… 
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