16 Questions
What is the primary function of the fibril-associated collagens?
To link collagen fibrils together
What is the role of hydroxylation in collagen synthesis?
To modify lysine and proline residues after incorporation into polypeptide chains
What is the function of glycine in the collagen triple helix?
To facilitate the formation of kinks in the peptide chain
What is the characteristic structure of collagen?
Elongated, triple-helical structure
What is the role of proline in the collagen triple helix?
To cause kinks in the peptide chain, facilitating the formation of the triple helix
What is the first step in the biosynthesis of collagen?
Transcription of genes of pro-alpha 1 and pro-alpha 2 chains
What is the function of lysyl hydroxylase in collagen production?
To hydroxylate selected proline and lysine residues
What is the characteristics of type 1 osteogenesis imperfecta (OI)?
Characterized by mild bone fragility, hearing loss, and blue sclera
What is the role of vitamin C in collagen production?
To hydroxylate proline and lysine residues
What is the difference between elastin and collagen?
Elastin is rich in glycine, alanine, valine, proline, and lysine, while collagen is rich in hydroxylysine and hydroxyproline
What is the function of alpha1-antitrypsin (AAT)?
To inhibit elastase released during phagocytosis by neutrophils in alveoli of lungs
What is the characteristic of classic Ehlers-Danlos syndrome (EDS)?
Characterized by skin extensibility and fragility, and joint hypermobility
What is the consequence of a deficiency of alpha1-antitrypsin (AAT)?
Emphysema and cirrhosis
What is the role of procollagen peptidases in collagen production?
To cleave the N-terminal and C-terminal propeptides from procollagen
What is the characteristic of Marfan syndrome?
Characterized by impaired structural integrity in skeleton, eye, and cardiovascular system
What is the function of collagenases in collagen degradation?
To cleave collagen at specific sites, generating 3/4 and 1/4 length fragments
Test your knowledge of fibrous proteins, the most abundant protein in the human body, including their structure, types, and tissue distribution. Learn about fibril-forming, network-forming, and fibril-associated proteins.
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