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3-hydroxy-- 3-methylglutaryl-coa Lyase Deficiency
3-hydroxy-- 3-methylglutaryl-coa Lyase Deficiency
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Inborn errors of metabolism
(107)
2,4 Dienoyl-CoA reductase deficiency
2-Methylbutyryl-CoA dehydrogenase deficiency
3 methylcrotonyl-coa carboxylase deficiency
3-hydroxy-- 3-methylglutaryl-CoA lyase deficiency
3-hydroxya- cyl-coenzyme A dehydrogenase deficiency
3-hydroxya- cyl-coenzyme A dehydrogenase deficiency
Aciduria
Argininosuccinic acidemia
Beta-ketothiolase deficiency
Carbamoyl phosphate synthetase I deficiency
Citrullinemia
D-Glyceric acidemia
Glutaric acidemia type I
Hawkinsinuria
Holocarboxylase synthetase deficiency
Hypermethioninemia
Isobutyryl-CoA dehydrogenase deficiency
Isovaleric acidemia
LCHAD
Maple syrup urine disease
Medium chain acyl CoA dehydrogenase deficiency
Methylmalonic acidemia
N-acetylglutamate synthase deficiency
Newborn screening
PKU
Propionic acidemia
Sarcosinemia
Tetrahydrobiopterin deficiency
Trifunctional protein deficiency
Type I tyrosinemia
Type III tyrosinemia
Tyrosinemia
Tyrosinemia type II
Urocanic aciduria
Very-long-chain acyl-CoA dehydrogenase deficiency
Acatalasia
Acid lipase disease
Adenosine deaminase deficiency
Adenylosuccinate lyase deficiency
Aldolase A deficiency
Analbuminaemia
Arakawa's syndrome II
Argininemia
Aspartylgl- ucosaminuria
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
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
Hereditary fructose intolerance
Histidinemia
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
Menkes disease
Metab-L
Methylmalonyl-CoA mutase deficiency
Mevalonate kinase deficiency
Myoadenylate deaminase deficiency
Niemann-Pick disease, type C
Ornithine transcarbamylase deficiency
Ornithine translocase deficiency
Primary carnitine deficiency
Prolidase deficiency
Pyruvate carboxylase deficiency
Pyruvate kinase deficiency
Refsum's disease
Rotor syndrome
Schindler disease
Short-chain acyl-coenzyme A dehydrogenase deficiency
Sly syndrome
Smith-Lemli-Opitz syndrome
Succinic semialdehyde dehydrogenase deficiency
Tangier disease
Trimethylaminuria
Urea cycle disorder
Wolman disease
X-linked ichthyosis
Zellweger syndrome
more...
Genetic disorders
(41)
3 methylglutaconyl coa hydratase deficiency
Hypertryptophanemia
2-Hydroxyglutaric aciduria
6-pyruvoyl- tetrahydropterin synthase deficiency
Pipecolic acidemia
Pipecolic acidemia
Hartnup disease
Homocystinuria
Hermansky-Pudlak syndrome
3-hydroxy-- 3-methylglutaryl-CoA lyase deficiency
Isobutyryl-CoA dehydrogenase deficiency
3-hydroxya- cyl-coenzyme A dehydrogenase deficiency
2-Methylbutyryl-CoA dehydrogenase deficiency
2,4 Dienoyl-CoA reductase deficiency
Type III tyrosinemia
Tyrosinemia type II
D-Glyceric acidemia
Type I tyrosinemia
3 methylcrotonyl-coa carboxylase deficiency
Hypermethioninemia
Citrullinemia
Urocanic aciduria
Trifunctional protein deficiency
Hawkinsinuria
Very-long-chain acyl-CoA dehydrogenase deficiency
LCHAD
N-acetylglutamate synthase deficiency
Glutaric acidemia type I
Isovaleric acidemia
Argininosuccinic acidemia
Beta-ketothiolase deficiency
Aciduria
Holocarboxylase synthetase deficiency
Tetrahydrobiopterin deficiency
Medium chain acyl CoA dehydrogenase deficiency
Maple syrup urine disease
Propionic acidemia
Sarcosinemia
Carbamoyl phosphate synthetase I deficiency
Tyrosinemia
Methylmalonic acidemia
PKU
more...
Genetic disorder
(9)
2-Methylbutyryl-CoA dehydrogenase deficiency
2,4 Dienoyl-CoA reductase deficiency
Trifunctional protein deficiency
Hawkinsinuria
6-pyruvoyl- tetrahydropterin synthase deficiency
6-pyruvoyl- tetrahydropterin synthase deficiency
Pipecolic acidemia
Sarcosinemia
Carbamoyl phosphate synthetase I deficiency
Methylmalonic acidemia
more...
Rare diseases
(14)
Hypertryptophanemia
2-Hydroxyglutaric aciduria
Urocanic aciduria
Newborn screening
Pipecolic acidemia
Pipecolic acidemia
Isovaleric acidemia
Argininosuccinic acidemia
Beta-ketothiolase deficiency
Hartnup disease
Maple syrup urine disease
Propionic acidemia
Sarcosinemia
Methylmalonic acidemia
Hermansky-Pudlak syndrome
more...
Biochemistry
3-methylglutaconyl CoA
Metabolic
Carnitine
Autosomal recessive disorders
(251)
17-beta-hy- droxysteroid dehydrogenase deficiency
2-Hydroxyglutaric aciduria
3 methylcrotonyl-coa carboxylase deficiency
Abdallat Davis Farrage syndrome
Abderhalde- n-Kaufmann-Lignac syndrome
Abderhalde- n-Kaufmann-Lignac syndrome
Abetalipop- roteinemia
Ablepharon macrostomia syndrome
Acatalasia
Aceruloplasminemia
Acheiropodia
Acrocallosal syndrome
Acrodermatitis enteropathica
Acute fatty liver of pregnancy
Adducted thumb syndrome
Adenine phosphorib- osyltransferase deficiency
Adenosine deaminase deficiency
Adenylosuccinate lyase deficiency
Aicardi-Goutieres syndrome
Aldolase A deficiency
Alkaptonuria
Alpha-mannosidosis
Antley-Bixler syndrome
Argininemia
Argininosuccinic acidemia
Arterial tortuosity syndrome
Aspartylgl- ucosaminuria
Atelosteogenesis, type II
Atransferrinemia
Bare lymphocyte syndrome
Batten disease
Behr's syndrome
Bernard-Soulier syndrome
Beta-ketothiolase deficiency
Beta-mannosidosis
Bietti's crystalline dystrophy
Biotinidase deficiency
Bloom syndrome
Blue diaper syndrome
CAMFAK syndrome
Canavan disease
Carbamoyl phosphate synthetase I deficiency
Carnitine palmitoylt- ransferase I deficiency
Carnitine palmitoylt- ransferase II deficiency
Carnitine-- acylcarnitine translocase deficiency
Carnosinemia
Carpenter syndrome
Cartilage–hair hypoplasia
Cenani Lenz syndactylism
Cerebrotendineous xanthomatosis
Chediak–Higashi syndrome
Chondrodystrophy
Chorea acanthocytosis
Citrullinemia
Cockayne syndrome
Compound heterozygosity
Congenital adrenal hyperplasia
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Congenital hepatic fibrosis
Congenital ichthyosiform erythroderma
Congenital insensitivity to pain with anhidrosis
Craniodiaphyseal dysplasia
Cystathioninuria
Cystic fibrosis
Cystinosis
Cystinuria
D-Glyceric acidemia
DOOR syndrome
Diastrophic dysplasia
Dihydropyrimidine dehydrogenase deficiency
Donohue syndrome
Dubin-Johnson syndrome
Dubowitz syndrome
EAST syndrome
EEM syndrome
Ellis-van Creveld syndrome
Essential fructosuria
Ethylmalonic encephalopathy
Familial Mediterranean fever
Familial dysautonomia
Familial isolated vitamin E deficiency
Fanconi anemia
Farber disease
Fibrochondrogenesis
Finnish heritage disease
Fountain syndrome
Fraser syndrome
Friedreich's ataxia
Fucosidosis
Fumarase deficiency
GM2-gangliosidosis, AB variant
Galactokinase deficiency
Galactose epimerase deficiency
Galactose-- 1-phosphate uridylyltransferase galactosemia
Galloway Mowat syndrome
Gangliosidosis
Gastroschisis
Gaucher's disease
Gerodermia osteodysplastica
Giant axonal neuropathy
Gitelman syndrome
Glanzmann's thrombasthenia
Glucose-galactose malabsorption
Glutaric acidemia type 2
Glutaric acidemia type I
Glutathione synthetase deficiency
Glycogen storage disease type I
Glycogen storage disease type II
Glycogen storage disease type III
Glycogen storage disease type V
Griscelli syndrome
Guanidinoacetate methyltransferase deficiency
Gunther disease
Harlequin type ichthyosis
Hartnup disease
Hemophagocytic lymphohistiocytosis
Hereditary pyropoikilocytosis
Hermansky-Pudlak syndrome
Histidinemia
Holocarboxylase synthetase deficiency
Homocystinuria
Hurler syndrome
Hyperlysinemia
Hypermethioninemia
Hyperprolinemia
Hypertryptophanemia
Hypervalinemia
ICF syndrome
Ichthyosis lamellaris
Iminoglycinuria
Impossible syndrome
Infantile free sialic acid storage disease
Infantile neuroaxonal dystrophy
Isobutyryl-CoA dehydrogenase deficiency
Isovaleric acidemia
Jervell and Lange-Nielsen syndrome
Johanson-Blizzard syndrome
Juvenile Primary Lateral Sclerosis
Kaufman oculocerebrofacial syndrome
Keutel syndrome
Kindler syndrome
Krabbe disease
LCHAD
Lafora disease
Laron syndrome
Lecithin cholesterol acyltransferase deficiency
Leukocyte adhesion deficiency
Lipid storage disorder
Lipoid congenital adrenal hyperplasia
Lucey-Driscoll syndrome
Lysinuric protein intolerance
Malonyl-CoA decarboxylase deficiency
Maple syrup urine disease
Meckel syndrome
Medium chain acyl CoA dehydrogenase deficiency
Meleda disease
Metachromatic leukodystrophy
Methemoglobinemia
Methylmalonic acidemia
Mevalonate kinase deficiency
Micro syndrome
Microvillous inclusion disease
Morquio syndrome
Mucolipidosis
Mucolipidosis type IV
Mucopolysa- ccharidosis
Multiple sulfatase deficiency
N-acetylglutamate synthase deficiency
Nemaline myopathy
Nephronophthisis
Netherton syndrome
Neuronal ceroid lipofuscinosis
Nezelof syndrome
Niemann-Pick disease, type C
Niemann–Pick disease
Ochronosis
Oculodentodigital syndrome
Oguchi disease
Omenn syndrome
Ornithine translocase deficiency
Orotic aciduria
Otospondyl- omegaepiphyseal dysplasia
PKU
Papillon–Lefèvre syndrome
Pendred syndrome
Persistent Mullerian duct syndrome
Phosphofructokinase deficiency
Primary carnitine deficiency
Primary ciliary dyskinesia
Progressive external ophthalmoplegia
Prolidase deficiency
Propionic acidemia
Pseudodominance
Pseudoxanthoma elasticum
Purine nucleoside phosphorylase deficiency
Pycnodysostosis
Pyruvate carboxylase deficiency
Rabson-Mendenhall syndrome
Raine syndrome
Rapadilino syndrome
Recessive multiple epiphyseal dysplasia
Renal dysplasia-limb defects syndrome
Renal-hepa- tic-pancreatic dysplasia
Reproductive compensation
Restrictive dermopathy
Richner Hanhart syndrome
Rothmund–Thomson syndrome
Rotor syndrome
Sabinas brittle hair syndrome
Saccharopinuria
Salla disease
Sandhoff disease
Sanfilippo syndrome
Sarcosinemia
Short-chain acyl-coenzyme A dehydrogenase deficiency
Shwachman-Diamond syndrome
Sickle cell trait
Sickle-cell disease
Situs inversus
Sly syndrome
Smith-Lemli-Opitz syndrome
Succinic semialdehyde dehydrogenase deficiency
Sugarman syndrome
Survival motor neuron spinal muscular atrophy
Tangier disease
Tay-Sachs disease
Tetrahydrobiopterin deficiency
Thalassemia
Trifunctional protein deficiency
Trimethylaminuria
Triosephosphate isomerase deficiency
Type I tyrosinemia
Type III tyrosinemia
Tyrosinemia
Tyrosinemia type II
Urbach–Wiethe disease
Urocanic aciduria
Usher syndrome
Vaso-occlusive crisis
Very-long-chain acyl-CoA dehydrogenase deficiency
Vici syndrome
Weissenbac- her-Zweymüller syndrome
Werner syndrome
Wilson's disease
Wolcott-Rallison syndrome
Wolman disease
Woodhouse-Sakati syndrome
Xeroderma pigmentosum
Yunis-Varon syndrome
ZAP70 deficiency
Zazam Sheriff Phillips syndrome
Zunich–Kaye syndrome
more...
Diseases and disorders
(30)
Inborn errors of renal tubular transport
Thiolase deficiency
Fatty acid oxidation disorders
Acidaemia
Albinism
Albinism
Hyperammonemia
Iodine deficiency
Hypertryptophanemia
Tyrosinemia type II
D-Glyceric acidemia
2-Hydroxyglutaric aciduria
Citrullinemia
Urocanic aciduria
Trifunctional protein deficiency
Hawkinsinuria
6-pyruvoyl- tetrahydropterin synthase deficiency
Pipecolic acidemia
Argininosuccinic acidemia
Beta-ketothiolase deficiency
Aciduria
Holocarboxylase synthetase deficiency
Hartnup disease
Maple syrup urine disease
Sarcosinemia
Carbamoyl phosphate synthetase I deficiency
Tyrosinemia
Methylmalonic acidemia
Homocystinuria
PKU
Hermansky-Pudlak syndrome
more...
See also
(14)
HMG-CoA lyase
Isovaleryl-CoA dehydrogenase
HMG-CoA synthase
Acyl CoA dehydrogenase
Anatomy
Anatomy
Chemical substances
HMG-CoA
Objects
Hydroxyzine
Urea cycle
Amino acid metabolism
Lyase
Hydroxysteroid dehydrogenase
Leucine
more...
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