Esophageal smooth muscle dysfunction in oculopharyngeal muscular dystrophy

1996 ◽  
Vol 41 (7) ◽  
pp. 1350-1354 ◽  
Author(s):  
E. Timony ◽  
O. Khilkevic ◽  
A. D. Korczyn ◽  
R. Kimmel ◽  
A. Hallak ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Muscular Dystrophy ◽  
Oculopharyngeal Muscular Dystrophy ◽  
Muscle Dysfunction ◽  
Smooth Muscle Dysfunction

Prevalence of smooth muscle dysfunction among children with Duchenne muscular dystrophy

2020 ◽  
Vol 62 (6) ◽  
pp. 699-704
Author(s):  
Ranjith K. Manokaran ◽  
Sandeep Aggarwala ◽  
Rakesh Kumar ◽  
Arun K. Gupta ◽  
Biswaroop Chakrabarty ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Muscle Dysfunction ◽  
Smooth Muscle Dysfunction

scholarly journals Homeostatic and therapeutic roles of VIP in smooth muscle function: myo-neuroimmune interactions

2009 ◽  
Vol 297 (4) ◽  
pp. G716-G725 ◽  
Author(s):  
Xuan-Zheng Shi ◽  
Sushil K. Sarna
Keyword(s):  
Smooth Muscle ◽  
Inflammatory Response ◽  
Muscle Function ◽  
Contractile Response ◽  
Enteric Neurons ◽  
Muscle Dysfunction ◽  
Spontaneous Release ◽  
Circular Smooth Muscle ◽  
Smooth Muscle Function ◽  
Smooth Muscle Dysfunction

We tested the hypothesis that spontaneous release of vasoactive intestinal peptide (VIP) from enteric neurons maintains homeostasis in smooth muscle function in mild inflammatory insults and that infusion of exogenous VIP has therapeutic effects on colonic smooth muscle dysfunction in inflammation. In vitro experiments were performed on human colonic circular smooth muscle tissues and in vivo on rats. The incubation of human colonic circular smooth muscle strips with TNF-α suppressed their contractile response to ACh and the expression of the pore-forming α1C subunit of Cav1.2 channels. VIP reversed both effects by blocking the translocation of NF-κB to the nucleus and its binding to the κB recognition sites on hα1C1b promoter. The translocation of NF-κB was inhibited by blocking the degradation of IκBβ. Induction of inflammation by a subthreshold dose of 17 mg/kg trinitrobenzene sulfonic acid (TNBS) in rats moderately decreased muscularis externa concentration of VIP, and it had little effect on the contractile response of circular smooth muscle strips to ACh. The blockade of VIP and pituitary adenylate cyclase-activating peptide receptors 1/2 during mild inflammatory insult significantly worsened the suppression of contractility and the inflammatory response. The induction of more severe inflammation by 68 mg/kg TNBS induced marked suppression of colonic circular muscle contractility and decrease in serum VIP. Exogenous infusion of VIP by an osmotic pump reversed these effects. We conclude that the spontaneous release of VIP from the enteric motor neurons maintains homeostasis in smooth muscle function in mild inflammation by blocking the activation of NF-κB. The infusion of exogenous VIP mitigates colonic inflammatory response and smooth muscle dysfunction.

scholarly journals Smooth muscle dysfunction occurs independently of impaired endothelium-dependent dilation in adults at risk of atherosclerosis

1998 ◽  
Vol 32 (1) ◽  
pp. 123-127 ◽  
Author(s):  
Mark R Adams ◽  
Jacqui Robinson ◽  
Robyn McCredie ◽  
J.Paul Seale ◽  
Keld E Sorensen ◽  
...  
Keyword(s):  
At Risk ◽  
Smooth Muscle ◽  
Muscle Dysfunction ◽  
Smooth Muscle Dysfunction

scholarly journals High-resolution iris and retinal imaging in multisystemic smooth muscle dysfunction syndrome due to a novel Asn117Lys substitution in ACTA2: a case report

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Aisling B. Mc Glacken-Byrne ◽  
David Prentice ◽  
Danial Roshandel ◽  
Michael R. Brown ◽  
Philip Tuch ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Case Report ◽  
High Resolution ◽  
Retinal Imaging ◽  
Muscle Dysfunction ◽  
Smooth Muscle Dysfunction

MR Imaging Diagnosis of ACTA2

Neurographics ◽  
2019 ◽  
Vol 9 (6) ◽  
pp. 409-413
Author(s):  
R. Farias-Moeller ◽  
S.M. Lew ◽  
R. Sacho ◽  
T.G. Kelly
Keyword(s):  
Smooth Muscle ◽  
Clinical Manifestations ◽  
Muscle Dysfunction ◽  
Imaging Features ◽  
Imaging Diagnosis ◽  
Childhood Stroke ◽  
Intestinal Dysmotility ◽  
Prognostic Implications ◽  
Smooth Muscle Dysfunction ◽  
Large Vessel Disease

Smooth muscle alpha-2 actin (ACTA2) mutations are associated with diffuse smooth-muscle dysfunction syndrome and produce distinct imaging features. Clinical manifestations include intrathoracic large-vessel disease, nonreactive mydriasis, bladder hypotonia, and intestinal dysmotility. ACTA2 mutations are associated with childhood stroke due to diffuse cerebral arteriopathy distinct from moyamoya disease. Recognition of this syndrome has important clinical and prognostic implications. The purpose of this article is to review the unique imaging phenotypes associated with ACTA2 mutations, including vascular and nonvascular abnormalities.

scholarly journals Bladder smooth muscle dysfunction in patients with irritable bowel syndrome.

Gut ◽  
1986 ◽  
Vol 27 (9) ◽  
pp. 1014-1017 ◽  
Author(s):  
P J Whorwell ◽  
E W Lupton ◽  
D Erduran ◽  
K Wilson
Keyword(s):  
Irritable Bowel Syndrome ◽  
Smooth Muscle ◽  
Muscle Dysfunction ◽  
Bladder Smooth Muscle ◽  
Irritable Bowel ◽  
Smooth Muscle Dysfunction

scholarly journals An HDAC9-MALAT1-BRG1 complex mediates smooth muscle dysfunction in thoracic aortic aneurysm

2018 ◽  
Vol 9 (1) ◽  
Author(s):  
Christian L. Lino Cardenas ◽  
Chase W. Kessinger ◽  
Yisha Cheng ◽  
Carolyn MacDonald ◽  
Thomas MacGillivray ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Aortic Aneurysm ◽  
Thoracic Aortic Aneurysm ◽  
Muscle Dysfunction ◽  
Smooth Muscle Dysfunction
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