title: Visceral smooth muscle α-actin deficiency associated with chronic intestinal pseudo-obstruction in a Bengal cat (Felis catus x Prionailurus bengalensis).
abstract: An adult Bengal cat (Felis catus × Prionailurus bengalensis) with a prolonged history of partial anorexia, regurgitation, and weight loss and a clinical, radiographic, and ultrasonographic diagnosis of persistent megaesophagus and gastrointestinal ileus was submitted for necropsy. The intestinal tract was diffusely distended by gas and fluid with appreciable loss of muscle tone and an absence of luminal obstruction, consistent with the clinical history of chronic intestinal pseudo-obstruction. Histologically, the autonomic nervous system was intact, but the smooth muscle within the gastrointestinal wall exhibited a marked basophilia that was most pronounced in the jejunum. Immunohistochemistry for neurofilament, synaptophysin, CD117, and desmin demonstrated that the number of myenteric ganglia, number of interstitial cells, and leiomyocyte desmin content were similar when compared with the unaffected age- and species-matched control. Immunohistochemistry for smooth muscle α-actin demonstrated a striking loss of immunoreactivity, predominantly in the circular layer of the jejunum, that corresponded with the tinctorial change in leiomyocytes. Transmission electron microscopy revealed loss of myofibrils, loss of organelle polarity, and significantly larger central mitochondria (megamitochondria) in affected leiomyocytes, as well as nonspecific degenerative changes. Although the presence of a primary leiomyopathy and a causal relationship could not be confirmed in this case, leiomyopathies are considered a cause of chronic intestinal pseudo-obstruction in human medicine, and loss of smooth muscle α-actin immunoreactivity is one recognized marker for intestinal dysmotility.
authors.affiliation: Department of Pathobiological Sciences, School of Veterinary Medicine, University of Wisconsin, 2015 Linden Dr West, Madison, WI 53706, USA. Email: steinbeh@svm.vetmed.wisc.edu.
authors.forename: D M
authors.initials: DM
authors.lastname: Imai
authors.forename: J L
authors.initials: JL
authors.lastname: Miller
authors.forename: B C
authors.initials: BC
authors.lastname: Leonard
authors.forename: J
authors.initials: J
authors.lastname: Bach
authors.forename: R
authors.initials: R
authors.lastname: Drees
authors.forename: H
authors.initials: H
authors.lastname: Steinberg
authors.forename: L B C
authors.initials: LB
authors.lastname: Teixeira
mesh_terms: D000199:Actins / Q000172:deficiency* / Q000378:metabolism; D000818:Animals; D002371:Cat Diseases / Q000473:pathology*; D002415:Cats / Q000235:genetics*; D003677:Deficiency Diseases / Q000150:complications / Q000473:pathology / Q000662:veterinary*; D003893:Desmin / Q000378:metabolism; D045989:Felidae / Q000235:genetics*; D006824:Hybridization, Genetic / Q000235:genetics*; D007150:Immunohistochemistry / Q000662:veterinary; D007418:Intestinal Pseudo-Obstruction / Q000209:etiology / Q000662:veterinary*; D046529:Microscopy, Electron, Transmission / Q000662:veterinary; D008928:Mitochondria / Q000473:pathology; D032389:Myocytes, Smooth Muscle / Q000378:metabolism; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism; D016708:Synaptophysin / Q000378:metabolism
chemical_list: D000199:Actins; D003893:Desmin; D016708:Synaptophysin; D019009:Proto-Oncogene Proteins c-kit
keywords: chronic intestinal pseudo-obstruction; dysautonomia; feline; ileus; smooth muscle α-actin; visceral leiomyopathy