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2-methylbutyryl-coa Dehydrogenase Deficiency
2-Methylbutyryl-CoA dehydrogenase deficiency
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Inborn errors of metabolism
(106)
3-MCC
3-hydroxy-- 3-methylglutaryl-CoA lyase deficiency
3-hydroxya- cyl-coenzyme A dehydrogenase deficiency
Citrullinemia
Glutaric acidemia type I
Glutaric acidemia type I
Hypermethioninemia
Isobutyryl-CoA dehydrogenase deficiency
Isovaleric acidemia
LCHAD
Maple syrup urine disease
Newborn screening
Propionic acidemia
Trifunctional protein deficiency
Tyrosinemia type II
Very-long-chain acyl-CoA dehydrogenase deficiency
2,4 Dienoyl-CoA reductase deficiency
Acatalasia
Acid lipase disease
Adenosine deaminase deficiency
Adenylosuccinate lyase deficiency
Aldolase A deficiency
Analbuminaemia
Arakawa's syndrome II
Argininemia
Argininosuccinic aciduria
Aspartylgl- ucosaminuria
Beta-ketothiolase deficiency
Carbamoyl phosphate synthetase I deficiency
Carnitine palmitoylt- ransferase I deficiency
Carnitine palmitoylt- ransferase II deficiency
Carnitine-- acylcarnitine translocase deficiency
Cholesteryl ester storage disease
Congenital disorder of glycosylation
Crigler-Najjar syndrome
D-Glyceric acidemia
DOOR syndrome
Dihydropyrimidine dehydrogenase deficiency
Disaccharidase
Essential fructosuria
Fucosidosis
Fumarase deficiency
Galactose-- 1-phosphate uridylyltransferase galactosemia
Galactosemia
Glucose-galactose malabsorption
Glutaric acidemia type 2
Glycogen storage disease
Glycogen storage disease type I
Glycogen storage disease type II
Glycogen storage disease type III
Glycogen storage disease type V
Guanidinoacetate methyltransferase deficiency
Hawkinsinuria
Hereditary fructose intolerance
Histidinemia
Holocarboxylase synthetase deficiency
Hyperlysinemia
Hyperprolinemia
Hypervalinemia
Idiosyncrasy
Inborn error of lipid metabolism
Inborn error of metabolism
Krabbe disease
Lecithin cholesterol acyltransferase deficiency
Lesch–Nyhan syndrome
Lipid storage disorder
Lipid storage disorders
Lysinuric protein intolerance
Lysosomal storage disease
Lysosomal storage diseases
Malonyl-CoA decarboxylase deficiency
Medium-chain acyl-coenzyme A dehydrogenase deficiency
Menkes disease
Metab-L
Methylmalonic acidemia
Methylmalonyl-CoA mutase deficiency
Mevalonate kinase deficiency
Myoadenylate deaminase deficiency
N-Acetylglutamate synthase deficiency
Niemann-Pick disease, type C
Organic acidemia
Ornithine transcarbamylase deficiency
Ornithine translocase deficiency
Phenylketonuria
Primary carnitine deficiency
Prolidase deficiency
Pyruvate carboxylase deficiency
Pyruvate kinase deficiency
Refsum's disease
Rotor syndrome
Sarcosinemia
Schindler disease
Short-chain acyl-coenzyme A dehydrogenase deficiency
Sly syndrome
Smith-Lemli-Opitz syndrome
Succinic semialdehyde dehydrogenase deficiency
Tangier disease
Tetrahydrobiopterin deficiency
Trimethylaminuria
Type I tyrosinemia
Type III tyrosinemia
Tyrosinemia
Urea cycle disorder
Urocanic aciduria
Wolman disease
X-linked ichthyosis
Zellweger syndrome
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Genetic disorders
(16)
3 methylglutaconyl coa hydratase deficiency
2-Hydroxyglutaric aciduria
Isobutyryl-CoA dehydrogenase deficiency
3-hydroxy-- 3-methylglutaryl-CoA lyase deficiency
3-hydroxya- cyl-coenzyme A dehydrogenase deficiency
3-hydroxya- cyl-coenzyme A dehydrogenase deficiency
Tyrosinemia type II
Very-long-chain acyl-CoA dehydrogenase deficiency
3-MCC
Glutaric acidemia type I
Trifunctional protein deficiency
LCHAD
Isovaleric acidemia
Propionic acidemia
Hypermethioninemia
Citrullinemia
Maple syrup urine disease
more...
Autosomal recessive disorders
(13)
Isobutyryl-CoA dehydrogenase deficiency
Tyrosinemia type II
Very-long-chain acyl-CoA dehydrogenase deficiency
2-Hydroxyglutaric aciduria
3-MCC
3-MCC
Glutaric acidemia type I
Trifunctional protein deficiency
LCHAD
Isovaleric acidemia
Propionic acidemia
Hypermethioninemia
Citrullinemia
Maple syrup urine disease
more...
Rare diseases
2-Hydroxyglutaric aciduria
Isovaleric acidemia
Propionic acidemia
Maple syrup urine disease
Newborn screening
Diseases and disorders
(9)
Thiolase deficiency
Fatty acid oxidation disorders
Iodine deficiency
Mental retardation
Tyrosinemia type II
Tyrosinemia type II
2-Hydroxyglutaric aciduria
Trifunctional protein deficiency
Citrullinemia
Maple syrup urine disease
more...
Enzyme
Isovaleryl-CoA dehydrogenase
3-hydroxy-- 2-methylbutyryl-CoA dehydrogenase
3-Hydroxyacyl CoA dehydrogenase
Dehydrogenase
Enzymes
Acyl CoA dehydrogenase
Hydroxysteroid dehydrogenase
Dehydrogenase
Genetic disorder
Trifunctional protein deficiency
See also
2-Methylbutyryl-CoA
HSD17B10
Chemical substances
Objects
Mitochondrial
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