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Fibrosis quística: alteración de la secreción de bicarbonato y mucoviscidosis
1Department of Pediatrics, University of California, San Diego School of Medicine, and Biomedical Sciences, University of California, Riverside (UCR), La Jolla, CA 92093, USA. pquinton@ucsd.edu
Lancet (London, England)
|August 5, 2008
Resumen
El engrosamiento del moco de la fibrosis quística puede deberse a un transporte defectuoso de bicarbonato, no solo a problemas en los canales de cloruro. Esto afecta la expansión y el transporte de la mucina, lo que sugiere un doble papel en la patogénesis de la enfermedad.
Área de la Ciencia:
- La bioquímica es la bioquímica.
- Fisiología Fisiología Fisiología.
- Genética La genética.
Sus antecedentes:
- La fibrosis quística se caracteriza por un moco anormalmente grueso (mucoviscidosis).
- Esto se ha relacionado con defectos genéticos en el canal de cloruro del regulador de conductividad transmembrana (CFTR) de la fibrosis quística.
- Las teorías actuales atribuyen la deshidratación al transporte de fluidos defectuoso dependiente del cloruro, pero esto es fisiológicamente inconsistente.
Objetivo del estudio:
- Proponer una nueva hipótesis para la patogénesis de la fibrosis quística.
- Investigar el papel del transporte de bicarbonato (HCO3-) en la expansión de la mucina y la formación de moco.
- Desafiar la dependencia exclusiva de los defectos de transporte de cloruro para explicar la fibrosis quística.
Principales métodos:
- Revisión de los datos fisiológicos y genéticos existentes sobre la fibrosis quística.
- Análisis de los mecanismos de liberación y expansión de los gránulos de mucina.
- La hipótesis del papel del bicarbonato en la complejación catiónica para la solubilización de la mucina.
Principales resultados:
- La expansión de la mucina requiere la eliminación de los cationes Ca2+) y H2+).
- El bicarbonato (HCO3-) es crucial para la expansión normal de la mucina al formar complejos con estos cationes.
- La secreción defectuosa de HCO3 en la fibrosis quística conduce a mucinas mal expandidas y agregadas.
Conclusiones:
- La patogénesis de la fibrosis quística puede implicar el transporte defectuoso de bicarbonato tanto como el transporte de cloruro.
- El deterioro de la expansión y solubilización de la mucina debido a los defectos de HCO3 contribuye a la enfermedad.
- Esta hipótesis ofrece una nueva perspectiva sobre la fisiopatología de la fibrosis quística.
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