Type III Collagen Quiz

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Questions and Answers

Which gene mutation in mice led to premature death at 13-14 weeks?

COL3A1 gene mutation causing severe skin wounds

Defective COL3A1 gene in tight skin mouse (Tsk2/+)

Transgenic mice with a Gly182Ser mutation (correct)

Naturally occurring large deletion of the COL3A1 gene

Which mouse model exhibited a malformation of the brain?

Tight skin mouse (Tsk2/+)

Mice with a naturally occurring large deletion of the COL3A1 gene (correct)

Mice with defective COL3A1 gene

Transgenic mice with a Gly182Ser mutation

Which mouse model resembled the human systemic sclerosis?

Transgenic mice with a Gly182Ser mutation

Mice with defective COL3A1 gene causing severe skin wounds

Mice with a naturally occurring large deletion of the COL3A1 gene

Tight skin mouse (Tsk2/+) (correct)

Which type of mice died suddenly due to thoracic aortic dissections?

Mice with a naturally occurring large deletion of the COL3A1 gene Signup and view all the answers

What is the composition of Type III Collagen?

It is a homotrimer, composed of three identical peptide chains Signup and view all the answers

How is the structure of Type III Collagen stabilized?

By the formation of disulphide bonds Signup and view all the answers

What post-translational modification occurs to the lysine residues of Type III Collagen?

They can be hydroxylated or glycosylated Signup and view all the answers

What is the result of the folding of individual chains of Type III Collagen?

They fold into left-handed helix and are wrapped together into a right-handed superhelix Signup and view all the answers

What is the primary role of type III collagen in tissues?

Providing tensile strength and forming macromolecular fibrils and fibers in the extracellular space Signup and view all the answers

What is the structural reason for the triple-helical conformation of type III collagen?

Presence of glycine as every third amino acid in the sequence Signup and view all the answers

Where is type III collagen primarily found alongside type I collagen?

Various tissues Signup and view all the answers

Which gene is responsible for type III collagen?

COL3A1 Signup and view all the answers

What severe disease can result from mutations in the COL3A1 gene?

Vascular Ehlers-Danlos syndrome (vEDS) Signup and view all the answers

What is the consequence of about 2/3 of the mutations in the COL3A1 gene?

Changing a glycine amino acid to another amino acid in the triple-helical region of the protein chain Signup and view all the answers

What is the functional consequence of COL3A1 mutations that can be studied in a cell culture system?

Using skin fibroblasts that express type III collagen Signup and view all the answers

Where are increased amounts of type III collagen found in fibrotic conditions?

Liver and kidney Signup and view all the answers

What is the consequence of inactivation of the murine COL3A1 gene in mouse models?

Leading to a shorter life span in homozygous mutant mice Signup and view all the answers

What role does type III collagen play in blood clotting?

Facilitates platelet aggregation through its binding to platelets Signup and view all the answers

What is the widely used biomarker for diagnosing fibrotic conditions?

N-terminal propeptide of type III procollagen Signup and view all the answers

Study Notes

Type III Collagen: Structure, Function, and Clinical Significance

Type III collagen is a major component of tissues requiring tensile strength, forming macromolecular fibrils and fibers in the extracellular space.
The triple-helical conformation of type III collagen is due to the presence of glycine as every third amino acid in the sequence of about 1000 amino acids.
Type III collagen is found in hollow organs such as large blood vessels, uterus, and bowel, as well as in various tissues alongside type I collagen.
The COL3A1 gene, responsible for type III collagen, is located on chromosome 2 and has 51 exons, and its mutations can lead to severe diseases such as vascular Ehlers-Danlos syndrome (vEDS).
Mutations in the COL3A1 gene cause vEDS, the most severe form of Ehlers-Danlos syndrome, leading to sudden death due to the rupture of large arteries or other hollow organs.
Over 600 different mutations have been characterized in the COL3A1 gene, with about 2/3 of them changing a glycine amino acid to another amino acid in the triple-helical region of the protein chain.
Type III collagen is also implicated in severe brain anomalies, and it is a known ligand for the receptor GRP56.
Functional consequences of COL3A1 mutations can be studied in a cell culture system using skin fibroblasts that express type III collagen.
Increased amounts of type III collagen are found in fibrotic conditions such as liver and kidney fibrosis, and systemic sclerosis, leading to a search for serum biomarkers for diagnosing these conditions.
Four different mouse models with COL3A1 defects have been reported, with inactivation of the murine COL3A1 gene leading to a shorter life span in homozygous mutant mice.
Type III collagen has implications for blood clotting as it facilitates platelet aggregation through its binding to platelets.
The N-terminal propeptide of type III procollagen, cleaved off during the biosynthesis of type III collagen, is a widely used biomarker for diagnosing fibrotic conditions.

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Description

Test your knowledge about Type III collagen, its structure, function, and clinical significance. Explore its role in tissues, genetic mutations, associated diseases, and its implications for diagnosing fibrotic conditions.