Perlecan, CollagenXVIII, and Agrin Expression in Normo-, Hypo-, and Aganglionic Segments in Hirschsprung's Disease.

ABSTRACT:

Background

Secretory heparan sulphate proteoglycans (HSPGs) interact with various morphogens, growth factors, and signaling molecules contributing to the development of the enteric nervous system (ENS). Thus, HSPGs have come into focus as pathomechanistic players of enteric neuropathies, such as Hirschsprung's disease (HSCR) in animal models. However, a detailed description of HSPG expression in human HSCR patients is missing.

Methods

We characterized the expression pattern of the secretory HSPGs perlecan, COL18A1, and agrin in the human ENS and investigated differences in affected and healthy intestinal segments. Thus, comparative immunostainings were performed on human gut samples from HSCR-patients and on non-HSCR controls as well as tissues from human body donors.

Key

SUBMITTER: van den Beld N 

PROVIDER: S-EPMC12816820 | biostudies-literature | 2026 Jan

REPOSITORIES: biostudies-literature