title: Intestinal pseudo-obstruction with deficient smooth muscle alpha-actin.
abstract: We describe a 48-year-old woman with chronic constipation since early childhood who has an intestinal myopathy associated with a hitherto undescribed absence by immunostaining of smooth muscle alpha-actin confined to the intestinal circular muscle. There were no abnormalities in other contractile proteins (myosin, tropomyosin, filamin, caldesmon or desmin) and despite the abnormality of a contractile protein isoform in the circular muscle, no significant morphological changes were identified by light microscopy or ultrastructural examination. A possible developmental mechanism for the observed change is proposed. The use of specific antibodies to isoforms of contractile proteins may have potential value in the study of intestinal myopathies.
authors: Hospital for Sick Children, Department of Histopathology, London, UK.
authors: V V
authors: VV
authors: Smith
authors: B D
authors: BD
authors: Lake
authors: M A
authors: MA
authors: Kamm
authors: R J
authors: RJ
authors: Nicholls
mesh_terms: D000199:Actins / Q000032:analysis*; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D007418:Intestinal Pseudo-Obstruction / Q000378:metabolism* / Q000473:pathology*; D007583:Jejunum / Q000737:chemistry / Q000473:pathology; D008875:Middle Aged
chemical_list: D000199:Actins