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El gen knockout humano CLYBL conecta el itaconato con la vitamina B12

Hongying Shen¹, Gregory C Campanello², Daniel Flicker¹

¹Howard Hughes Medical Institute and Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA; Broad Institute, Cambridge, MA 02141, USA.

|October 24, 2017

Resumen

La enzima CLYBL actúa como una citramalyl-CoA liasa, afectando el metabolismo de la vitamina B12. Su ausencia provoca la acumulación de citramil-CoA y altera los procesos mitocondriales dependientes de la vitamina B12.

Área de la Ciencia:

La bioquímica
Metabolomía
Biología estructural

Sus antecedentes:

CLYBL es una enzima mitocondrial conservada con función desconocida.
La pérdida de CLYBL conduce a una reducción de los niveles de vitamina B12.
El itaconato es un metabolito inmunomodulador vinculado a la vía del C5-dicarboxilato.

Objetivo del estudio:

Para aclarar la actividad celular y la vía metabólica de CLYBL.
Investigar el vínculo entre CLYBL, el metabolismo de la vitamina B12 y el itaconato.
Caracterizar el mecanismo del papel de CLYBL en las células de mamíferos.

Principales métodos:

Enzimología y técnicas de biología estructural.
Metabolomía basada en la actividad.
Análisis de las células con deficiencia de CLYBL.

Principales resultados:

CLYBL funciona como una citramalyl-CoA liasa en las células de los mamíferos.
La deficiencia de CLYBL provoca la acumulación de citramil-CoA.
El itaconil-CoA inhibe la metilmalonil-CoA mutasa (MUT), que es dependiente de la vitamina B12.

Conclusiones:

Se identifica la función de CLYBL como citramalyl-CoA liasa.
La pérdida de CLYBL causa un defecto celular autónomo en el metabolismo mitocondrial de la vitamina B12.
El metabolito inmunomodulador itaconato inactiva las enzimas dependientes de la vitamina B12.

Palabras clave:

Metabolismo de las C5 CLYBL (en inglés)Citramalil-CoA liasa y sus derivados genética humana el itaconato el metabolismo Las mitocondrias Biología de sistemas Vitamina B y sus derivados (12)

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