RESUMEN

In this study, a total of 11 species belonging to four genera of Myzininae are recorded from China, among which a new species, namely Hylomesa punctata sp. nov. is described from Tibet and Yunnan, and four species are newly recorded. All the species are illustrated and a key to them is provided in this paper.

Asunto(s)

Himenópteros , Distribución Animal , Animales , China

RESUMEN

AIM: To determine whether pirfenidone attenuates lung fibrosis by interfering with the hedgehog (Hh) signalling pathway in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: Twenty-five SSc-ILD patients (20 first visit, five who underwent pirfenidone treatment for 6 months) and 10 healthy controls were recruited. Lung tissues were obtained by open-chest surgery, and primary lung fibroblasts were isolated, cultured and stimulated with pirfenidone. The levels of the proteins glioma-associated oncogene 1 (GLI1), suppressor of fused (Sufu), α-smooth muscle actin, and fibronectin in lung tissues or fibroblasts were determined by Western blotting. The messenger RNA levels of GLI1, glioma-associated oncogene 2, protein patched homolog 1, and Sufu in lung tissues or fibroblasts were determined by quantitative reverse-transcription polymerase chain reaction. Meanwhile, the levels of phosphorylation glycogen synthase kinasep-3ß (pGSK-3ß), phosphorylation SMAD2 (pSMAD2), and phosphorylation c-Jun N-terminal kinase (pJNK) in fibroblasts were determined by Western blotting. RESULTS: Hh pathway activation was increased in the lung tissue of SSc-ILD patients and was decreased by pirfenidone, Sufu was upregulated in lung fibroblasts isolated from SSc-ILD patients after pirfenidone challenge, and pirfenidone inhibited the phosphorylation of GSK-3ß signalling. CONCLUSION: Pirfenidone has anti-fibrotic effects in SSc-ILD patients by interfering with both the Hh signalling pathway and the GSK-3ß signalling pathway via the regulation of Sufu expression. These results might promote its use in other Hh driven lung diseases such as idiopathic pulmonary fibrosis and especially the interstitial lung disease associated with connective tissue diseases.

Asunto(s)

Fibroblastos/efectos de los fármacos , Proteínas Hedgehog/metabolismo , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Pulmón/efectos de los fármacos , Fibrosis Pulmonar/tratamiento farmacológico , Piridonas/farmacología , Esclerodermia Sistémica/tratamiento farmacológico , Transducción de Señal/efectos de los fármacos , Actinas/metabolismo , Adulto , Estudios de Casos y Controles , Células Cultivadas , Femenino , Fibroblastos/metabolismo , Fibroblastos/patología , Fibronectinas/metabolismo , Glucógeno Sintasa Quinasa 3 beta/metabolismo , Humanos , Pulmón/metabolismo , Pulmón/patología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/metabolismo , MAP Quinasa Quinasa 4/metabolismo , Masculino , Persona de Mediana Edad , Proteínas Nucleares/genética , Proteínas Nucleares/metabolismo , Receptor Patched-1/genética , Receptor Patched-1/metabolismo , Fosforilación , Fibrosis Pulmonar/diagnóstico , Fibrosis Pulmonar/metabolismo , Proteínas Represoras/genética , Proteínas Represoras/metabolismo , Esclerodermia Sistémica/diagnóstico , Esclerodermia Sistémica/metabolismo , Proteína Smad2/metabolismo , Tomografía Computarizada por Rayos X , Proteína con Dedos de Zinc GLI1/genética , Proteína con Dedos de Zinc GLI1/metabolismo , Proteína Gli2 con Dedos de Zinc/genética , Proteína Gli2 con Dedos de Zinc/metabolismo