72 Questions
Which type of proteins have a long cylindrical shape and low solubility in water?
Fibrous proteins
What is a common characteristic of fibrous proteins?
Regular secondary structure
Which of the following is an example of a fibrous protein?
Collagen
Which organ/tissue has collagen fibers that are arranged at an angle to each other to resist mechanical shear from any direction?
Bone
How many distinct types of collagen are found in the human body?
More than 25
What is the main function of collagen in the human body?
To provide structural strength
Which type of collagen is a major component of the basal lamina?
Type IV
What is the repeating banding pattern observed in collagen fibrils?
64 nm
Which type of collagen is involved in Classical Ehlers Danlos Syndrome?
Type V
Which type of collagen is found in the dermis, bone, tendon, dentin, fascias, sclera, organ capsules, and fibrous cartilage?
Type I
Which type of collagen is involved in the structural maintenance of expansible organs such as smooth muscle, endoneurium, arteries, uterus, liver, spleen, kidney, and lung?
Type III
Which type of collagen is a major component of the basal lamina in epithelial and endothelial tissues?
Type IV
Which amino acid is frequently found in the X position of the repeating triplets in collagen?
Proline
What is the primary role of glycine in the collagen helix?
To provide flexibility to the helix
How many residues are there per turn in the helices of collagen?
3
Which amino acid is responsible for conferring rigidity to the collagen triple helix?
Hydroxyproline
Which amino acid is involved in H-bond formation that helps to stabilize the collagen triple helix?
Hydroxyproline
Which amino acid is the site of attachment of carbohydrate moieties in collagen?
Hydroxylysine
Which modification is essential for enhancing the stability and function of collagen chains?
Hydroxylation
What facilitates the assembly of collagen chains into a triple helical structure?
Molecular chaperones
Where are the mature collagen molecules secreted into?
Extracellular matrix
Which of the following is required for the post-translational hydroxylation of proline and lysine in collagen synthesis?
Molecular oxygen, iron, and Vitamin C
Which process involves the glycosylation of hydroxylysine residues in collagen synthesis?
O glycosidic bonds to galactose and glucose
What is the role of Vitamin C (Ascorbate) in collagen synthesis?
It is required for the post-translational hydroxylation of proline and lysine
Which direction does the winding of the triple helix occur in collagen formation?
From the carboxyl terminus towards the amino terminus
What initiates the winding of the triple helix in collagen formation?
Folding of carboxy-terminal globular domains
What is the order of events in collagen formation?
Formation of disulfide bonds, folding of carboxy-terminal globular domains, winding of the triple helix
Which organelle is responsible for the release of completed procollagen from the cell?
Golgi apparatus
What is the role of N- and C- procollagen peptidases in collagen formation?
They remove the terminal pro-peptides, releasing the triple helix tropocollagen molecule
How is completed procollagen released from the cell?
Via exocytosis
Which enzyme catalyzes the conversion of lysine residues into its aldehyde derivative allysine in collagen fibrils?
Lysyl oxidase
What is the primary role of cross-linking in the fibrillar array of collagen molecules?
To enhance the tensile strength
What is the function of lysyl oxidase in collagen synthesis?
To promote the formation of cross-links
Which of the following is a consequence of the overlapping individual triple strands in collagen fibril formation?
Regular axial periodic pattern
What is the key characteristic of the mesoscopic structures formed during collagen fibril formation?
Micron length
What is the significance of the staggered arrangement of collagen triple strands in fibril formation?
Contributes to D-banding pattern
Which enzyme is responsible for the degradation of collagen during tissue remodeling or in response to growth/injury of tissue?
Collagenases
What happens to collagen during degradation?
It is broken into smaller fragments
What is the effect of a mutation that interferes with the ability of collagen to form cross-linked fibrils?
It almost certainly affects the stability of collagen
Which of the following is NOT a function of collagen in maintaining homeostasis?
Initiating the formation of blood clots
What is the role of collagen in wound healing?
Serving as a scaffold for cellular migration
How does collagen contribute to vascular health?
Providing structural support to arterial walls
Which type of collagen is primarily affected in Classical Ehlers Danlos Syndrome?
Type V collagen
Which type of collagen is primarily affected in the Vascular type of Ehlers Danlos Syndrome?
Type III collagen
Which enzyme is responsible for the pathogenesis of the Kyphoscoliotic type of Ehlers Danlos Syndrome?
Lysyl hydroxylase
Which type of Ehlers Danlos Syndrome is the most common?
Hypermobile EDS (hEDS)
What is the genetic basis for Classical EDS (cEDS)?
COL5A1, COL5A2
Which type of collagen is primarily affected in Vascular EDS (vEDS)?
Type III collagen
Which type of Osteogenesis Imperfecta is characterized by fractures secondary to minor trauma in infancy?
Type I - Osteogenesis imperfecta tarda
Which type of Osteogenesis Imperfecta is more severe and results in death in utero or neonatally from pulmonary hypoplasia?
Type II - Osteogenesis imperfecta congenita
Which type of Osteogenesis Imperfecta is characterized by similar mutations at the Gly position, causing deformities but not lethality?
Type III - Osteogenesis imperfecta
Which of the following is a symptom of scurvy, a condition resulting from vitamin C deficiency?
All of the above
Which protein gives tissues and organs the capacity to stretch without tearing?
Elastin
Where is elastin found in the body?
All of the above
Which statement about collagen is correct?
Collagen has a repeating structure of (Gly-X-Y)n.
What is the primary function of elastin in the human body?
To allow stretching of tissues without tearing.
Where is tropoelastin synthesized in the cell?
In the rough endoplasmic reticulum.
Which protein is responsible for elastogenesis?
Fibrillin
What is the molecular weight of elastin?
64 - 66 kDa
Which cross-link is unique to elastin?
Desmosine
Which of the following characteristics is NOT exhibited by mature, extracellular elastin?
Has a very low turnover rate
What is the primary function of mature, extracellular elastin?
To provide structural support to organs and tissues
Which of the following is a key characteristic of the conformation of mature, extracellular elastin?
Random coil conformations
Which enzyme is responsible for the degradation of elastin in the extracellular space?
Neutrophil elastase
What is the clinical significance of emphysema in individuals with genetic absence of α1-antitrypsin?
Emphysema is more severe in these individuals
What is the primary symptom of emphysema?
Shortness of breath
Which of the following is NOT a characteristic of α-keratins?
They are found in the outer epidermal layer of mammals
What is the result of the tandem repetition of a 7-residue segment in α-keratins?
Formation of a super-helical structure containing 2 polypeptide chains
What is the role of the polar edge in α-keratins?
Interacts with water on the outside of the superhelix
Which structure is formed when two keratin α chains combine in a coiled coil formation?
Dimer
How many protofilaments combine to form a filament (protofibril)?
4
What is the primary cause of changes in hair wave appearance during permanent waving?
Breakage of disulfide bonds
Test your knowledge about fibrous proteins and their characteristics such as their long cylindrical shape, low solubility in water, and structural role in cells. Explore examples like collagen, elastin, and keratin.
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