Up-regulated FHL1 expression maybe involved in the prognosis of Hirschsprung's disease.

ABSTRACT:

Background

In a subset of patients with Hirschsprung's disease (HSCR), gastrointestinal motor dysfunction persisted long after surgical correction. Gastrointestinal motility is achieved through the coordinated activity of the enteric nervous system, interstitial cells of Cajal, and smooth muscle (SMC) cells. Inhibition of four-and-a-half LIM protein-1 (Fhl1) expression by siRNA significantly decreases pulmonary artery SMCs migration and proliferation. Furthermore when up-expressing FHL1 in atrial myocytes, K (+) current density markedly increases, therefore changing myocytes' response to an electrical stimulus. However whether FHL1 in colon SMCs (the final effector organ) influences intestinal motility in HSCR patients has not been clarified.

Methods

FHL1 mRNA and protein ex

SUBMITTER: Wang LL 

PROVIDER: S-EPMC3917115 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature