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Myelin Protein Zero Immunohistochemistry Is Not a Reliable Marker of Extrinsic Mucosal Innervation in Patients With Hirschsprung Disease.
Kapur, Raj P; Tisoncik-Go, Jennifer; Gale, Michael.
Afiliación
  • Kapur RP; Department of Laboratory Medicine and Pathology, 7274Seattle Children's Hospital and the University of Washington, Seattle, WA, USA.
  • Tisoncik-Go J; Center for Innate Immunity and Immune Disease, Department of Immunology, 7284University of Washington, Seattle, WA, USA.
  • Gale M; Center for Innate Immunity and Immune Disease, Department of Immunology, 7284University of Washington, Seattle, WA, USA.
Pediatr Dev Pathol ; 25(4): 388-396, 2022.
Article en En | MEDLINE | ID: mdl-34904460
ABSTRACT

BACKGROUND:

Innervation of aganglionic rectum in Hirschsprung disease derives from extrinsic nerves which project from cell bodies located outside the bowel wall and markers that distinguish extrinsic from intrinsic innervation are diagnostically useful. Myelin protein zero (MPZ) is a putative marker of extrinsic glial cells which could distinguish mucosal innervation in aganglionic vs ganglionic colon.

METHODS:

Sections and protein blots from ganglionic and aganglionic colon were immunolabeled with MPZ-specific antibodies.

RESULTS:

Immunolabeling of MPZ with a chicken polyclonal or mouse monoclonal antibody confirmed glial specificity and reliably labeled hypertrophic submucosal nerves in Hirschsprung disease. In contrast, a rabbit polyclonal antibody strongly labeled extrinsic and intrinsic nerves, including most mucosal branches. Immunoblots showed MPZ is expressed in mucosal glial cells, albeit at lower levels than in extrinsic nerves, and that the rabbit antibody is more sensitive that the other two probes. Unfortunately, none of these antibodies consistently distinguished mucosal innervation in aganglionic vs ganglionic rectum.

CONCLUSIONS:

The results suggest that (a) glial cell myelin protein zero expression is influenced more by location (mucosa vs submucosa) than the extrinsic vs intrinsic origin of the accompanied nerves and (b) myelin protein zero immunohistochemistry has limited value as a diagnostic adjunct for Hirschsprung disease.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Hirschsprung Límite: Animals / Humans Idioma: En Revista: Pediatr Dev Pathol Asunto de la revista: PATOLOGIA / PEDIATRIA Año: 2022 Tipo del documento: Article País de afiliación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Hirschsprung Límite: Animals / Humans Idioma: En Revista: Pediatr Dev Pathol Asunto de la revista: PATOLOGIA / PEDIATRIA Año: 2022 Tipo del documento: Article País de afiliación: Estados Unidos