mnohočetná karboxylázová deficience, novorozenecká forma [Holocarboxylase Synthetase Deficiency]

tematický
Termíny

deficit synthetasy holokarboxylas
deficit synthetázy holokarboxyláz
mnohočetná karboxylasová deficience infantilní
mnohočetná karboxylasová deficience novorozenecká
mnohočetný karboxylázový deficit, novorozenecká forma
synthetasa holokarboxylas - nedostatek

 

Carboxylase Deficiency, Multiple, Neonatal Form
Deficiency, Holocarboxylase Synthetase
Deficiency, Multiple Carboxylase, Neonatal Form
Early-Onset Biotin-Responsive Multiple Carboxylase Deficiency
Early-Onset Combined Carboxylase Deficiency
HLCS Deficiency
Infantile Multiple Carboxylase Deficiency
Multiple Carboxylase Deficiency, Early Onset
Multiple Carboxylase Deficiency, Neonatal Form

Perzistentní odkaz   https://www.medvik.cz/link/D028922
Definice

Novorozenecká forma mnohočetné karboxylázové deficience, která je vyvolaná deficitem syntetázy holokarboxyláz. Tento enzym kovalentně váže biotin ke karboxylázám závislým na biotinu (propionyl-CoA-karboxyláza, pyruvátkarboxyláza a beta-metylkrotonyl-CoA-karboxyláza).

The neonatal form of MULTIPLE CARBOXYLASE DEFICIENCY that is caused by a defect or deficiency in holocarboxylase synthetase. HLCS is the enzyme that covalently links biotin to the biotin dependent carboxylases (propionyl-CoA-carboxylase, pyruvate carboxylase, and beta-methylcrotonyl-CoA carboxylase).

DUI
D028922 MeSH Prohlížeč
CUI
M0381890
Historická pozn.
2002
Veřejná pozn.
2002