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Deranged smooth muscle alpha-actin as a biomarker of intestinal pseudo-obstruction: a controlled multinational case series

Knowles, CH; Silk, DBA; Darzi, A; Veress, Bela LU ; Feakins, R; Raimundo, AH; Crompton, T; Browning, EC; Lindberg, G and Martin, JE (2004) In Gut 53(11). p.1583-1589
Abstract
Background and aims: Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a severe motility disorder associated with significant morbidity. Several histopathological ( neuropathic and myopathic) phenotypes have been described but only a single adult with jejunal smooth ( circular) muscle alpha-actin deficiency. We present a prospective multinational case series investigating smooth muscle alpha-actin deficiency as a biomarker of this disease. Methods: A total of 115 fully clinically and physiologically ( including prolonged ( 24 hour) ambulatory jejunal manometry) characterised CIIP patients from three European centres were studied. Immunohistochemical localisation of actins and other cytoskeletal proteins were performed on... (More)
Background and aims: Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a severe motility disorder associated with significant morbidity. Several histopathological ( neuropathic and myopathic) phenotypes have been described but only a single adult with jejunal smooth ( circular) muscle alpha-actin deficiency. We present a prospective multinational case series investigating smooth muscle alpha-actin deficiency as a biomarker of this disease. Methods: A total of 115 fully clinically and physiologically ( including prolonged ( 24 hour) ambulatory jejunal manometry) characterised CIIP patients from three European centres were studied. Immunohistochemical localisation of actins and other cytoskeletal proteins were performed on laparoscopic full thickness jejunal biopsies and compared with adult controls. Distribution of alpha-actin was also characterised in other gut regions and in the developing human alimentary tract. Results: Twenty eight of 115 (24%) CIIP patient biopsies had absent (n = 22) or partial ( n = 6) jejunal smooth muscle alpha-actin immunostaining in the circular muscle layer. In contrast, smooth muscle alpha-actin staining was preserved in the longitudinal muscle and in adult jejunal controls ( n = 20). Comparative study of other adult alimentary tract regions and fetal small intestine, suggested significant spatial and temporal variations in smooth muscle alpha-actin expression. Conclusions: The ability to modulate alpha-smooth muscle actin expression, evident in development, is maintained in adult life and may be influenced by disease, rendering it a valuable biomarker even in the absence of other structural abnormalities. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Gut
volume
53
issue
11
pages
1583 - 1589
publisher
BMJ Publishing Group
external identifiers
  • pmid:15479676
  • wos:000224407200011
  • scopus:4344711479
ISSN
1468-3288
DOI
10.1136/gut.2003.037275
language
English
LU publication?
yes
id
af403b37-b2e7-42de-93a8-1022bd70bd52 (old id 898356)
date added to LUP
2008-01-15 13:10:42
date last changed
2017-09-24 04:19:45
@article{af403b37-b2e7-42de-93a8-1022bd70bd52,
  abstract     = {Background and aims: Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a severe motility disorder associated with significant morbidity. Several histopathological ( neuropathic and myopathic) phenotypes have been described but only a single adult with jejunal smooth ( circular) muscle alpha-actin deficiency. We present a prospective multinational case series investigating smooth muscle alpha-actin deficiency as a biomarker of this disease. Methods: A total of 115 fully clinically and physiologically ( including prolonged ( 24 hour) ambulatory jejunal manometry) characterised CIIP patients from three European centres were studied. Immunohistochemical localisation of actins and other cytoskeletal proteins were performed on laparoscopic full thickness jejunal biopsies and compared with adult controls. Distribution of alpha-actin was also characterised in other gut regions and in the developing human alimentary tract. Results: Twenty eight of 115 (24%) CIIP patient biopsies had absent (n = 22) or partial ( n = 6) jejunal smooth muscle alpha-actin immunostaining in the circular muscle layer. In contrast, smooth muscle alpha-actin staining was preserved in the longitudinal muscle and in adult jejunal controls ( n = 20). Comparative study of other adult alimentary tract regions and fetal small intestine, suggested significant spatial and temporal variations in smooth muscle alpha-actin expression. Conclusions: The ability to modulate alpha-smooth muscle actin expression, evident in development, is maintained in adult life and may be influenced by disease, rendering it a valuable biomarker even in the absence of other structural abnormalities.},
  author       = {Knowles, CH and Silk, DBA and Darzi, A and Veress, Bela and Feakins, R and Raimundo, AH and Crompton, T and Browning, EC and Lindberg, G and Martin, JE},
  issn         = {1468-3288},
  language     = {eng},
  number       = {11},
  pages        = {1583--1589},
  publisher    = {BMJ Publishing Group},
  series       = {Gut},
  title        = {Deranged smooth muscle alpha-actin as a biomarker of intestinal pseudo-obstruction: a controlled multinational case series},
  url          = {http://dx.doi.org/10.1136/gut.2003.037275},
  volume       = {53},
  year         = {2004},
}