Unit 06 Pt4
Overview of Intermediate Filaments
Focus on intermediate filaments as part of the cytoskeleton
Present only in some metazoan cells, unlike actin and microtubules which are ubiquitous
Diameter: 8-11 nanometers, rope-like structure conferring mechanical strength
Structure and Function
Intermediate filaments are important for cellular integrity and stress responses
Confers mechanical strength, supporting the structure of epithelial cells
Related to nuclear lamins, indicating diversity compared to actins and microtubules
Coiled-coil configuration mediated by alpha-helical domains in monomers
Formation: Dimers coalesce into antiparallel staggered tetramers
Key Features
Lack of nucleotide binding (no GTP or ATP)
No polarity in polymerization: no directionality in assembly
Up to 32 polypeptides can combine, providing significant tensile strength
Can stretch up to three times their length without breaking
Types of Intermediate Filaments
Keratins
Most diverse type: dozens of keratin types defined by heterodimeric filament subunits consisting of one acidic and one basic subunit
Strong networks formed through disulfide bonds, known as "tough as nails"
Certain keratins associated with carcinomas, aiding tissue origin identification in cancers
Epidermolysis Bullosa (EB)
A genetic disorder associated with keratin mutations
Characterized by skin blistering from minor mechanical stress
Result of inadequate tensile strength in intermediate filaments, leading to epithelial cells forming abnormal structures
Neurofilaments
Found specifically in neuron tissue, heteropolymers along axons
Expression levels affect axon diameter and electrical impulse transmission speed
Abnormalities linked with conditions like ALS due to accumulation of neurofilament assemblies
Linker Proteins and Their Functions
Example: Plectin connects intermediate filaments to actin and microtubules
Important for cellular adhesion and linking structures to the plasma membrane
Mutations in linker proteins associated with disorders like EB and muscular dystrophies
Nuclear Lamin Connection
SUN and Cache proteins bridge nuclear lamina and cytoskeleton
SUN domain proteins located in the inner nuclear membrane; Cache proteins in the outer membrane
Interactions facilitate connections between nuclear laminins and other components
Mutations can result in progerias and accelerated aging
Septins
GTP-binding proteins crucial for cell polarity and division
Assemble into nonpolar filaments forming rings and cages
Important for compartmentalizing membranes during cell division, migration, and vesicular trafficking
Contribute to the formation of primary cilia
Visuals included show septin functions in yeast cell division and vesicular transport