Related Experiment Video
Updated: Oct 7, 2025
07:12
Author Spotlight: Using Point-of-Care Ultrasound for Comprehensive Evaluation of the Abdominal Aorta
Published on: September 8, 2023
3.1K
Increased Risk of Aortic Dissection with Perlecan Deficiency
Risa Nonaka1,2,3, Takafumi Iesaki4,5, Aurelien Kerever1
1Research Institute for Diseases of Old Age, Juntendo University Graduate School of Medicine, Tokyo 113-8421, Japan.
International Journal of Molecular Sciences
|January 11, 2022
Summary
Perlecan deficiency in mice leads to aortic dissection due to immature elastic fibers and a weakened aortic wall. This study identifies perlecan as a crucial factor in maintaining aortic integrity.
Area of Science:
- Biochemistry
- Molecular Biology
- Cardiovascular Research
Background:
- Perlecan (HSPG2) is a basement membrane proteoglycan involved in tissue development.
- Its specific role in aortic wall development and maintenance is not fully understood.
- Perlecan deficiency is linked to a high incidence of aortic dissection (AD) in mice.
Purpose of the Study:
- To investigate the role of perlecan in the development and maintenance of the aortic wall.
- To elucidate the mechanisms by which perlecan deficiency contributes to aortic dissection.
- To propose a new model for AD pathogenesis involving perlecan deficiency.
Main Methods:
- Analysis of the aortic wall structure in perlecan-deficient (Perl KO) mice.
- Biochemical assessment of elastic fiber components (desmosine, tropoelastin).
- Evaluation of smooth muscle cell contractile protein expression (actin, myosin).
Main Results:
- Perlecan deficiency resulted in thinner, torn elastic laminae in the aortic wall.
- Perl KO mice exhibited decreased desmosine and increased soluble tropoelastin, indicating immature elastic fibers.
- Reduced expression of actin and myosin in perlecan-deficient aortic tissue was observed.
- Perlecan deficiency was associated with increased risk of aortic dissection.
Conclusions:
- Perlecan deficiency impairs aortic wall integrity, leading to increased risk of aortic dissection.
- Immaturity of the extracellular matrix, particularly elastic fibers, is a key factor in perlecan-deficiency-induced AD.
- Perlecan is identified as a novel risk factor in a proposed model of aortic dissection.
More Related Videos
05:31Murine Model of Thoracic Aortic Dissection Induced by Oral β-Aminopropionitrile and Subcutaneous Angiotensin II Infusion
Published on: May 16, 2025
377
06:46Quantitative Micro-CT Analysis of Aortopathy in a Mouse Model of β-aminopropionitrile-induced Aortic Aneurysm and Dissection
Published on: July 16, 2018
9.7K
Related Concept Videos
01:30Aneurysm I: Introduction
40
An aortic aneurysm is a localized outpouching or dilation at a weak point in the artery wall. It may involve different parts of the aorta, such as the abdominal aorta, aortic arch, or thoracic aorta.Etiological factorsSeveral disorders are associated with aortic aneurysms.Congenital causes, such as primary connective tissue disorders like Marfan syndrome, impact the integrity and strength of connective tissues, notably affecting the aorta. Marfan syndrome is a genetic disorder that specifically...
40
01:21Aneurysm II: Clinical Manifestations and Diagnostic Studies
44
Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
44
01:22Aortic Regurgitation II: Clinical Features and Diagnostic Tests
73
Aortic valve regurgitation (AR) occurs when the aortic valve fails to close properly, allowing blood to flow backward from the aorta into the left ventricle. This backflow can result in two distinct clinical presentations: acute and chronic AR, each characterized by its own set of symptoms and physical findings.Acute Aortic RegurgitationAcute AR presents with a sudden onset of severe symptoms. Patients typically experience profound dyspnea (shortness of breath), chest pain, and signs of left...
73
01:30Peripheral Artery Disease I: Introduction
63
Peripheral artery disease (PAD) predominantly results from atherosclerosis, which involves the accumulation of fatty deposits, or plaques, within the walls of arteries. This causes them to narrow and harden, significantly reducing blood flow. PAD predominantly affects the legs, particularly the arteries supplying the thighs and calves. In rare cases, it may involve other arteries, including those in the arms.Etiology of PAD:The principal cause of PAD is atherosclerosis, which results from fatty...
63
01:26Aneurysm III: Interprofessional Care
46
Aneurysm management involves either conservative medical therapy or surgical intervention, depending on the size and symptoms of the aneurysm. Conservative management is generally reserved for smaller, asymptomatic aneurysms, while larger or symptomatic aneurysms often necessitate surgical repair.Conservative Medical TherapyFor small, asymptomatic aneurysms, particularly abdominal aortic aneurysms (AAA) less than 5.5 centimeters in diameter, conservative medical therapy is recommended. This...
46
01:15Aortic Regurgitation I: Introduction
67
IntroductionAortic regurgitation is characterized by the backward flow of blood from the aorta into the left ventricle during diastole and arises from the improper closure of the aortic valve. This condition results in left ventricular volume overload and can stem from both acute and chronic etiologies, each contributing uniquely to the disease's progression and symptomatology.Acute and Chronic CausesAcute aortic regurgitation often results from events that suddenly impair the integrity of the...
67