Amino Acid Metabolism, Inborn Errors [vrozené poruchy metabolismu aminokyselin]

topical
124
Terms

aminokyseliny - vrozené poruchy metabolismu
dědičné poruchy metabolismu aminokyselin

 

Amino Acid Metabolism Disorders, Inborn
Amino Acid Metabolism, Inborn Error
Amino Acid Metabolism, Inherited Disorders
Amino Acidopathies, Congenital
Amino Acidopathies, Inborn
Congenital Amino Acidopathies
Inborn Errors, Amino Acid Metabolism
Inherited Errors of Amino Acid Metabolism

Persistent link   https://www.medvik.cz/link/D000592
Definition

Disorders affecting amino acid metabolism. The majority of these disorders are inherited and present in the neonatal period with metabolic disturbances (e.g., ACIDOSIS) and neurologic manifestations. They are present at birth, although they may not become symptomatic until later in life.

Annotation
coord IM with specific amino acid /metab (IM)
DUI
D000592 MeSH Browser
CUI
M0000918
History note
1965; PROTEIN METABOLISM DISORDERS was heading 1963-1964
Public note
1965; PROTEIN METABOLISM DISORDERS was heading 1963-1964

C Diseases
C16.320.565.100.102 Albinism 17
C16.320.565.100.187 Alkaptonuria 76
C16.320.565.100.477 Hyperglycinemia, Nonketotic 7
C16.320.565.100.480 Hyperhomocysteinemia 212
C16.320.565.100.544 Hyperlysinemias
C16.320.565.100.608 Maple Syrup Urine Disease 8
C16.320.565.100.766 Phenylketonurias 286
C16.320.565.100.794 Prolidase Deficiency
C16.320.565.100.823 Propionic Acidemia 3
C16.320.565.100.880 Tyrosinemias 18
C16.320.565.100.940 Urea Cycle Disorders, Inborn 7
C16.320.565.176 Amyloidosis, Familial 16
C16.320.565.595 Lysosomal Storage Diseases 73
C16.320.565.663 Peroxisomal Disorders 20
C16.320.565.753 Progeria 27
C18.452 Metabolic Diseases 1 200
C18.452.648.100.102 Albinism 17
C18.452.648.100.187 Alkaptonuria 76
C18.452.648.100.477 Hyperglycinemia, Nonketotic 7
C18.452.648.100.480 Hyperhomocysteinemia 212
C18.452.648.100.544 Hyperlysinemias
C18.452.648.100.608 Maple Syrup Urine Disease 8
C18.452.648.100.766 Phenylketonurias 286
C18.452.648.100.823 Propionic Acidemia 3
C18.452.648.100.880 Tyrosinemias 18
C18.452.648.100.940 Urea Cycle Disorders, Inborn 7
C18.452.648.176 Amyloidosis, Familial 16
C18.452.648.595 Lysosomal Storage Diseases 73
C18.452.648.663 Peroxisomal Disorders 20
C18.452.648.753 Progeria 27

2-Methylacetoacetyl CoA thiolase deficiency Disease MeSH Browser

2-Methylbutyryl-CoA Dehydrogenase Deficiency Disease MeSH Browser

3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency Disease MeSH Browser

3-Hydroxyisobutyric aciduria Disease MeSH Browser

5-oxoprolinase deficiency Disease MeSH Browser

Acidemia, isovaleric Disease MeSH Browser

Acyl-CoA Dehydrogenase Family, Member 9, Deficiency of Disease MeSH Browser

Adams Nance syndrome Disease MeSH Browser

Alaninuria with Microcephaly, Dwarfism, Enamel Hypoplasia, and Diabetes Mellitus Disease MeSH Browser

Alpha-ketoglutarate dehydrogenase deficiency Disease MeSH Browser

Amino Aciduria with Mental Deficiency, Dwarfism, Muscular Dystrophy, Osteoporosis, and Acidosis Disease MeSH Browser

Aminoacylase 1 deficiency Disease MeSH Browser

Arakawa syndrome 2 Disease MeSH Browser

Arginine-Glycine Amidinotransferase Deficiency Disease MeSH Browser

Aromatic amino acid decarboxylase deficiency Disease MeSH Browser

Beta ketothiolase deficiency Disease MeSH Browser

Beta-Aminoisobutyric Acid, Urinary Excretion of Disease MeSH Browser

Beta-Hydroxyisobutyryl CoA Deacylase Deficiency Disease MeSH Browser

Blue diaper syndrome Disease MeSH Browser

Camptodactyly taurinuria Disease MeSH Browser

Carboxypeptidase N Deficiency Disease MeSH Browser

Cystathionase Deficiency Disease MeSH Browser

Cysteine Peptiduria Disease MeSH Browser

Diaminopentanuria Disease MeSH Browser

Dibasic Amino Aciduria I Disease MeSH Browser

Dimethylglycine Dehydrogenase Deficiency Disease MeSH Browser

Gamma aminobutyric acid transaminase deficiency Disease MeSH Browser

Glucoglycinuria Disease MeSH Browser

Glutamate Monosodium Sensitivity Disease MeSH Browser

Glutamine deficiency, congenital Disease MeSH Browser

Glutaric Acidemia I Disease MeSH Browser

Glutaric Aciduria III Disease MeSH Browser

Glutathione synthetase deficiency Disease MeSH Browser

Glutathionuria Disease MeSH Browser

Glycinuria with or without Oxalate Urolithiasis Disease MeSH Browser

Histidinemia Disease MeSH Browser

Homocarnosinosis Disease MeSH Browser

Hydroxykynureninuria Disease MeSH Browser

Hydroxyprolinemia Disease MeSH Browser

Hyperleucine-Isoleucinemia Disease MeSH Browser

Hypermethioninemia Disease MeSH Browser

Hyperprolinemia type 2 Disease MeSH Browser

Hypertaurinuric Cardiomyopathy Disease MeSH Browser

Hypertryptophanemia, Familial Disease MeSH Browser

Ichthyosis, Split Hairs, and Amino Aciduria Disease MeSH Browser

Indolylacroyl Glycinuria with Mental Retardation Disease MeSH Browser

Isobutyryl-CoA dehydrogenase deficiency Disease MeSH Browser

Ketoadipicaciduria Disease MeSH Browser

Lysine Malabsorption Syndrome Disease MeSH Browser

Lysinuric Protein Intolerance Disease MeSH Browser

Mercaptolactate-Cysteine Disulfiduria Disease MeSH Browser

Methionine Malabsorption Syndrome Disease MeSH Browser

Methylmalonate Semialdehyde Dehydrogenase Deficiency Disease MeSH Browser

Methylmalonic Aciduria and Homocystinuria, CblD Type Disease MeSH Browser

Methylmalonic Aciduria and Homocystinuria, CblF Type Disease MeSH Browser

Methylmalonic Aciduria due to Methylmalonyl-CoA Mutase Deficiency Disease MeSH Browser

Methylmalonic acidemia Disease MeSH Browser

Methylmalonic aciduria cblA type Disease MeSH Browser

Methylmalonic aciduria cblB type Disease MeSH Browser

Methylmalonyl-CoA Epimerase Deficiency Disease MeSH Browser

Methylmalonyl-CoA Epimerase Deficiency with Sepiapterin Reductase Deficiency Disease MeSH Browser

Myopathy due to Malate-Aspartate Shuttle Defect Disease MeSH Browser

Richards-Rundle syndrome Disease MeSH Browser

Sarcosinemia Disease MeSH Browser

Sulfite oxidase deficiency Disease MeSH Browser

Tiglic acidemia Disease MeSH Browser

Tryptophanuria With Dwarfism Disease MeSH Browser

Tyrosinosis Disease MeSH Browser

Urocanase deficiency Disease MeSH Browser

Valinemia Disease MeSH Browser

succinic semialdehyde dehydrogenase deficiency Disease MeSH Browser