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Descriptor English: Propionyl-Coenzyme A Carboxylase
Descriptor Spanish: Propionil-Coenzima A Carboxilasa
Descriptor propionil-coenzima A-carboxilasa
Entry term(s) propionil-CoA carboxilasa
propionil-CoA-carboxilasa
Scope note: Carboxilasa mitocondrial que interviene en el catabolismo de los ácidos grasos de cadena impar y de los aminoácidos ramificados. La disfunción de la propionil-CoA-carboxilasa causa ACIDEMIA PROPIÓNICA, un trastorno metabólico hereditario. En las bacterias, es una carboxilasa dependiente del ATP en reacciones de fijación de CO2 en las que la propionil-CoA se convierte en (S)-metilmalonil-CoA.
Descriptor Portuguese: Propionil-Coenzima A Carboxilase
Descriptor French: Without translation
Entry term(s): Propionyl-CoA Carboxylase
Tree number(s): D08.811.464.257.050.500
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D000095562
Scope note: Mitochondrial carboxylase that is involved in the catabolism of odd chain fatty acids and branched amino acids. Dysfunction of propionyl-CoA carboxylase leads to the inherited metabolic disorder PROPIONIC ACIDEMIA. In bacteria it is an ATP-dependent carboxylase in CO2 fixation reactions where propionyl-CoA is converted to (S)-methylmalonyl-CoA.
Allowable Qualifiers: AD administration & dosage
AE adverse effects
AI antagonists & inhibitors
AN analysis
BI biosynthesis
BL blood
CF cerebrospinal fluid
CH chemistry
CL classification
CS chemical synthesis
DE drug effects
EC economics
GE genetics
HI history
IM immunology
IP isolation & purification
ME metabolism
PD pharmacology
PH physiology
PK pharmacokinetics
PO poisoning
RE radiation effects
SD supply & distribution
ST standards
TO toxicity
TU therapeutic use
UL ultrastructure
UR urine
Entry Combination: deficiency:Propionic Acidemia
deficiência:Acidemia Propiônica
deficiencia:Acidemia Propiónica
Registry Number: EC 6.4.1.3
Previous Indexing: Methylmalonyl-CoA Decarboxylase (1979-2023)
Public MeSH Note: 2024; see Methylmalonyl-CoA Decarboxylase 2004-2023
History Note: 2024; use Methylmalonyl-CoA Decarboxylase 2004-2023
DeCS ID: 60429
Unique ID: D000095562
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2024/01/01
Date of Entry: 2023/07/26
Revision Date: 2023/02/26
Propionyl-Coenzyme A Carboxylase - Preferred
Concept UI M000758637
Scope note Mitochondrial carboxylase that is involved in the catabolism of odd chain fatty acids and branched amino acids. Dysfunction of propionyl-CoA carboxylase leads to the inherited metabolic disorder PROPIONIC ACIDEMIA. In bacteria it is an ATP-dependent carboxylase in CO2 fixation reactions where propionyl-CoA is converted to (S)-methylmalonyl-CoA.
Preferred term Propionyl-Coenzyme A Carboxylase
Entry term(s) Propionyl-CoA Carboxylase



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