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Carnitina palmitoiltransferasa I

Carnitina palmitoiltransferasa 1A (hepática)
Estructuras disponibles
PDB

Buscar ortólogos: PDBe, RCSB

 Estructuras enzimáticas
Identificadores
Símbolos CPT1A (HGNC: 2328) CPT1
Identificadores
externos
Número EC 2.3.1.21
Locus Cr. 11 q13.1-13.5
Ortólogos
Especies
Humano Ratón
Entrez
1374
UniProt
P50416 n/a
RefSeq
(ARNm)
NM_001876 n/a
PubMed (Búsqueda)
[1]


PMC (Búsqueda)
[2]
Carnitina palmitoiltransferasa 1B (muscular)
Estructuras disponibles
PDB Buscar ortólogos: PDBe, RCSB
Identificadores
Símbolo CPT1B (HGNC: 2329)
Identificadores
externos
Locus Cr. 22 q13.3
Ortólogos
Especies
Humano Ratón
Entrez
1375
UniProt
Q92523 n/a
RefSeq
(ARNm)
NM_152245 n/a
Carnitina palmitoiltransferasa 1C
Estructuras disponibles
PDB Buscar ortólogos: PDBe, RCSB
Identificadores
Símbolo CPT1C (HGNC: 18540)
Identificadores
externos
Locus Cr. 19 q13.33
Ortólogos
Especies
Humano Ratón
Entrez
126129
UniProt
Q8TCG5 n/a
RefSeq
(ARNm)
NM_152359 n/a

La carnitina palmitoiltransferasa I (CPTI) es una enzima mitocondrial. En el músculo y otros tejidos no hepáticos, CPT1 está asociada con la membrana mitocondrial externa. La CPTI media el transporte de ácidos grasos de cadena larga a través de la membrana, uniéndolos a moléculas de carnitina. Este enzima puede ser inhibido por malonil-CoA.

La forma "CPTIA" está asociada con la deficiencia en carnitina palmitoiltransferasa I.


Acil-CoA desde el citosol hasta la matriz mitocondrial.

Una deficiencia en la acilcarnitina palmitoiltransferasa 1 provocaría una reducción en la oxidación de ácidos grasos.

Véase también

[editar]
  • Carnitina palmitoiltransferasa II
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Carnitina palmitoiltransferasa I
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