1

Introduction to the Visual System

The visual system takes in information from the environment in from the same embryonic germ cell layer. The iris is the most
the form of light and analyzes and interprets the data. This pro- anterior portion of the uvea, acting as a diaphragm to regulate the
cess of sight and visual perception involves a complex system of amount of light entering the pupil. Two iris muscles control the
structures, each of which is designed for a specific purpose. The shape and diameter of the pupil and are supplied by the autonomic
organization of each structure enables it to perform its intended nervous system. Continuous with the iris at its root is the ciliary
function. body, which produces the components of the aqueous humor and
The eye houses the elements that take in light rays and change contains the muscle that controls the shape of the lens. The pos-
the light to a neural signal. It is protected by the surrounding terior part of the uvea, the choroid, is an anastomosing network
bone and connective tissue of the orbit. The eyelids cover and of blood vessels with a dense capillary network. The choroid sur-
protect the anterior surface of the eye and contain glands that rounds the retina and supplies nutrients to the outer retinal layers.
produce the lubricating tear film. Muscles that attach to the The neural tissue of the retina, by complex biochemical pro-
outer coat of the eye control and direct the globe’s movement, cesses, changes light energy into a signal that can be transmitted
and the muscles of both eyes are coordinated to provide bin- along a neural pathway. The signal passes through the retina,
ocular vision. A network of blood vessels supplies nutrients, exits the eye through the optic nerve, and is transmitted to vari-
and a complex system of nerves provides sensory, motor, and ous parts of the brain for processing.
autonomic innervation to the eye and surrounding structures. Within the globe are three spaces: the anterior chamber, pos-
The neural signal that carries visual information passes through terior chamber, and vitreous chamber. The anterior chamber is
a complex and intricately designed pathway within the central bounded in front by the cornea and posteriorly by the iris and
nervous system, enabling an accurate view of the surrounding anterior surface of the lens. The posterior chamber lies behind
environment. This information, evaluated by a process called the iris. The lens lies within the posterior chamber, and the outer
visual perception, influences a myriad of decisions and activities. border of the posterior chamber is the ciliary body. The anterior
This book examines the macroscopic and microscopic anat- and posterior chambers are continuous with one another through
omy and physiology of the components in this complex system, the pupil, and both contain the aqueous humor, which is pro-
as well as the supporting structures. duced by the ciliary body. The aqueous humor provides nourish-
ment for the surrounding structures, particularly the cornea and
lens. The vitreous chamber, which is the largest space, lies adja-
ANATOMIC FEATURES OF THE EYE cent to the inner retinal layer and is bounded in front by the lens.
The eye, also called the globe, is a special sense organ made up This chamber contains a gel-like substance, the vitreous humor.
of three coats, or tunics (Fig. 1.1): The crystalline lens is located in the area of the posterior
1. The outer fibrous layer of connective tissue forms the cornea chamber and provides additional refractive power for accurately
and sclera. focusing images onto the retina. The lens must change shape to
2. The middle vascular layer is composed of the iris, ciliary view an object that is close to the eye through the mechanism of
body, and choroid. accommodation.
3. The inner neural layer is the retina.
The outer dense connective tissue of the eye offers protec-
tion for the structures within, maintains the shape of the globe,
ANATOMIC DIRECTIONS AND PLANES
and provides resistance to the pressure of the fluids inside. The Anatomy is an exacting science, and specific terminology is
sclera is the opaque white area of the eye and is covered by a basic to its discussion. The following anatomic directions should
transparent tissue, the conjunctiva. The transparent cornea, at be familiar (Fig. 1.2):
the anterior part of the globe, allows light rays to enter the globe • Anterior, or ventral: toward the front
and, by refraction, helps bring these light rays into focus on the • Posterior, or dorsal: toward the back
retina. The region at which the cornea transitions to sclera and • Superior, or cranial: toward the head
conjunctiva is the limbus. • Inferior, or caudal: away from the head
Inner to the sclera and cornea is a vascular layer of the eye, the • Medial: toward the midline
uvea. The uvea is made up of three structures, each having a sepa- • Lateral: away from the midline
rate but interconnected function. Some of the histological layers • Proximal: near the point of origin
are continuous throughout all three structures and are derived • Distal: away from the point of origin
1
2 CHAPTER 1  Introduction to the Visual System

Iris Cornea

Anterior chamber
External scleral sulcus

Corneoscleral border
Bulbar conjunctiva

Ciliary muscle

Ora
serrata
Pars plicata
Ciliary body

Pars plana

Medial
rectus

Lateral
rectus

Retina

Choroid
Fovea
Sclera

Lamina cribrosa

Dural sheath
Long posterior ciliary
artery
Optic nerve
Short posterior ciliary arteries

Fig. 1.1  Horizontal section of the globe showing major components.

The following planes are used in describing anatomic struc- Because the globe is a spherical structure, references to loca-
tures (Fig. 1.3): tions can sometimes be confusing. In references to anterior and
• Sagittal: vertical plane running from anterior to posterior posterior locations of the globe, the anterior pole (i.e., center
locations, dividing the structure into right and left sides. of the cornea) is the reference point. For example, the pupil is
• Midsagittal: sagittal plane through the midline, dividing the anterior to the ciliary body (see Fig. 1.1). When layers or struc-
structure into right and left halves. tures are referred to as inner or outer, the reference is to the
• Coronal or frontal: vertical plane running from side to entire globe unless specified otherwise. The point of reference
side, dividing the structure into anterior and posterior is the center of the globe, which would lie within the vitreous.
parts. For example, the retina is inner to the sclera (see Fig. 1.1). In
• Axial or transverse: horizontal plane, dividing the structure addition, the term sclerad is used to mean toward the sclera,
into superior and inferior parts. and vitread is used to mean toward the vitreous.
 CHAPTER 1  Introduction to the Visual System 3

Sagittal (median)
plane

Coronal (frontal)
plane

Posterior
Superior

Inferior
Anterior

Axial
(horizontal)
plane

Lateral

Medial

Proximal

Distal

Fig. 1.3  Anatomic planes. (From Palastanga N, Field D, Soames

R. Anatomy and Human Movement. Oxford, UK: Butterworth-
Heinemann; 1989.)

lens and cornea are too strong or, more likely, the eyeball is too
Fig. 1.2  Anatomic directions. (From Palastanga N, Field D, So- long, causing parallel light rays to focus in front of the retina
ames R. Anatomy and Human Movement. Oxford, UK: Butter- (Fig. 1.4C). Myopia can be corrected by placing a concave lens
worth-Heinemann; 1989.) in front of the eye, causing the incoming light rays to diverge.

OPHTHALMIC INSTRUMENTATION
REFRACTIVE CONDITIONS Various instruments are used to assess the health and function
If the refractive power of the optical components of the eye, of elements of the visual pathway and the supporting structures.
primarily the cornea and lens, correlates with the distances This section briefly describes some of these instruments and the
between the cornea, lens, and retina so that incoming parallel structures examined.
light rays come into focus on the retina, a clear image will be The curvature of the cornea is one of the factors that deter-
seen. This condition is called emmetropia (Fig. 1.4A). No cor- mine the corneal refractive power. A keratometer measures the
rection, such as glasses or contact lenses, is necessary for clear curvature of the central 3 to 4 mm of the anterior corneal surface
distance vision. In hyperopia (farsightedness), the distance and provides information about the power and the difference in
from the cornea to the retina is too short for the refractive power curvature between the principle meridians at that location. An
of the cornea and lens, thereby causing images to focus behind automated corneal topographer maps the corneal surface and
the retina (Fig. 1.4B). Hyperopia can be corrected by placing a gives an indication of the corneal curvature at selected points.
convex lens in front of the eye to increase the convergence of This instrument is an important adjunct in the fitting of contact
the incoming light rays. In myopia (nearsightedness), either the lenses in difficult cases.
4 CHAPTER 1  Introduction to the Visual System

retinal layers. OCT angiography detects motion of blood and

uses this to produce high resolution images of the retinal and
choroidal vasculature. This does not require the use of injectable
dyes, and the images can be obtained within seconds. Additional
instrumentation can allow visualization of corneal layers, cells,
and nerves and can aid in the differentiation of bacterial, viral,
parasitic, and fungal infection in corneal tissue.
A The visual field is the area that a person sees, including those
areas seen in the periphery. A perimeter is used to test the extent,
sensitivity, and completeness of this visual field. Computerized
perimeters provide extremely detailed maps of the visual field,
as well as statistical information on the reliability of the test and
the probabilities of any defects.
Neuroimaging techniques, such as magnetic resonance
imaging and computed tomography, allow increasingly detailed
B imaging of the globe, orbit, and visual pathway anatomy. These
images provide physiological and pathological information
never before available. Having a basic understanding of the nor-
mal anatomical appearance will aid in detecting pathology.

BASIC HISTOLOGICAL FEATURES

Because many of the anatomical structures are discussed in this
book at the histological level, this section briefly reviews basic
C human histology. Other details of tissues are addressed in the
Fig. 1.4  Refractive conditions. A, Emmetropia, in which paral- pertinent chapters.
lel light comes to a focus on the retina. B, Hyperopia, in which All body structures are made up of one or more of the four
parallel light comes to a focus behind the retina (dotted lines). basic tissues: epithelial, connective, muscle, and nervous tissue.
A convex lens is used to correct the condition and bring the A tissue is defined as a collection of similar cells that are special-
light rays into focus on the retina. C, Myopia, in which parallel ized to perform a common function.
light comes to a focus in front of retina (dotted lines). A concave
lens is used to correct the condition and bring the light rays into Epithelial Tissue
focus on the retina. (Courtesy Karl Citek, O.D., Pacific University
Epithelial tissue often takes the form of sheets of epithelial cells
College of Optometry, Forest Grove, Ore.)
that either cover the external surface of a structure or that line a
cavity. Epithelial cells lie on a basement membrane that attaches
The inside portion of the eye surrounding the vitreous cham- them to underlying connective tissue. The basement membrane
ber is called the fundus. This is examined using an ophthalmo- can be divided into two parts: the basal lamina, secreted by the
scope, which illuminates the interior of the eye with a bright light. epithelial cell, and the reticular lamina, a product of the under-
The retina, optic nerve head, and blood vessels can be assessed lying connective tissue layer. The free surface of the epithelial cell
and information about ocular and systemic health obtained. This is the apical surface, whereas the surface that faces underlying
is the only place in the body in which blood vessels can be viewed tissue or rests on the basement membrane is the basal surface.
directly and noninvasively. Various systemic diseases, such as dia- Epithelial cells are classified according to shape (Fig. 1.5).
betes, hypertension, and arteriosclerosis, can alter ocular vessels. Squamous cells are flat and platelike, cuboidal cells are of equal
To obtain a more complete view of the inside of the eye, topical height and width, and columnar cells are higher than wide.
drugs are administered to influence the iris muscles, causing the Epithelium consisting of a single layer of cells is referred to as
pupil to become enlarged, or mydriatic. A binocular indirect oph- simple: simple squamous, simple cuboidal, or simple columnar.
thalmoscope allows stereoscopic viewing of the fundus. Endothelium is the special name given to the simple squamous
The outside of the globe and the eyelids can be assessed with layer that lines certain cavities. Epithelium consisting of several
a biomicroscope. This combination of an illumination system layers is referred to as stratified and is described by the shape of
and a binocular microscope allows stereoscopic views of various the cells in the surface layer. Only the basal or deepest layer of
parts of the eye. Particularly beneficial is the view of the trans- cells is in contact with the basement membrane, and this layer
parent ocular structures, such as the cornea and lens. A number usually consists of columnar cells.
of auxiliary instruments can be used with the biomicroscope to Keratinized, stratified squamous epithelium has a surface layer
measure intraocular pressure and to view the interior of the eye. of squamous cells with cytoplasm that has been transformed into
Optical coherence tomography (OCT) uses light waves to a substance called keratin, a tough protective material relatively
noninvasively obtain a cross-sectional image of optical struc- resistant to mechanical injury, bacterial invasion, and water loss.
tures. It provides three-dimensional mapping of the retina and These keratinized surface cells constantly are sloughed off and
the optic nerve head and can measure the thickness of specific are replaced from the layers below where cell division takes place.
 CHAPTER 1  Introduction to the Visual System 5

Simple

Squamous Cuboidal Columnar

Stratified

Squamous nonkeratinized Cuboidal

Keratinized Columnar

Fig. 1.5  Types of epithelia. (From Gartner LP, Hiatt JL. Color Textbook of Histology. 3rd ed. Phila-
delphia: Saunders; 2007, p 87.)

Many epithelial cells are adapted for secretion and, when (Fig. 1.6). Glands can also be named according to the composi-
gathered into groups, are referred to as glands. Glands can be tion of their secretion: mucous, serous, or sebaceous.
classified according to the manner of secretion—exocrine glands
secrete through a duct onto the epithelial surface, whereas Connective Tissue
endocrine glands secrete directly into the bloodstream. Glands Connective tissue provides structure and support and fills the
can also be classified according to the process of secretion pro- space not occupied by other tissue. Types of connective tissue
duction—holocrine glands secrete complete cells laden with the include bone, muscle, tendons, blood, lymph, and adipose
secretory material; apocrine glands secrete part of the cell cyto- tissue. Connective tissue consists of cells, fibers, and ground
plasm in the secretion; and the secretion of merocrine glands substance. A combination of insoluble protein fibers within the
is a product of the cell without loss of any cellular components ground substance is called the extracellular matrix. Connective

A B C

Secretion
Disintegrating
cell and its
contents Intact cell
(secretion)

New cell

Pinched off
portion of cell
(secretion)
Fig. 1.6  Modes of glandular secretion. A, Holocrine. B, Merocrine. C, Apocrine. (From Gartner
LP, Hiatt JL. Color Textbook of Histology. 3rd ed. Philadelphia: Saunders; 2007, p 105.)
6 CHAPTER 1  Introduction to the Visual System

tissue can be classified as loose or dense. Loose connective tis- myelinated or unmyelinated. Myelinization improves impulse
sue has relatively fewer cells and fibers per area than dense con- conduction speed. Astrocytes have a number of functions,
nective tissue, in which the cells and fibers are tightly packed. including providing physical and metabolic support, maintain-
Dense connective tissue can be characterized as regular or irreg- ing extracellular homeostasis, and participating in the blood
ular on the basis of fiber arrangement. brain barrier. Microglial cells mediate the immune response in
Among the cells that may be found in connective tissue are the central nervous system. They possess phagocytic properties
fibroblasts (flattened cells that produce and maintain the fibers and increase in number in areas of damage or disease.
and ground substance), macrophages (phagocytic cells), mast cells
(which contain heparin and histamine), and fat cells. Connective BRIEF REVIEW OF HUMAN CELLULAR
tissue composed primarily of fat cells is called adipose tissue.
The fibers found in connective tissue include flexible collagen
PHYSIOLOGY
fibers with high tensile strength, delicate reticular fibers, and A cell membrane surrounds each cell and is composed of a
elastic fibers, which can undergo extensive stretching. Collagen double layer of hydrophilic lipids surrounding a hydrophobic
fibers are a major component of much of the eye’s connective intermediate area (Fig. 1.7). The two hydrophilic phospholipid
tissue. These fibers are composed of protein macromolecules layers face the aqueous solutions on both the inside (intracel-
of tropocollagen that have a coiled helix of three polypeptide lular area) and outside (extracellular area) of the cell. A hydro-
chains. The individual polypeptide chains can differ in their phobic fatty acid chain extending from each phospholipid layer
amino acid sequences, and the tropocollagen has a banded pat- projects toward the center of the membrane. Cholesterol mol-
tern because of the sequence differences. Collagen is separated ecules found in the central fatty acid portion decrease the mem-
into various types on the basis of such differences, and several brane’s permeability to water soluble molecules. Carbohydrates
types are components of ocular connective tissue structures. may form a glycocalyx coating on the extracellular cell mem-
The amorphous ground substance, in which the cells and brane. Protein molecules may be embedded in both surfaces of
fibers are embedded, consists of water bound to glycosamino- the lipid bilayer, and membrane-spanning proteins have por-
glycans, proteoglycans, and glycoproteins. tions both inside and outside the cell.
The cellular cytoplasm (cytosol) contains various protein
Muscle Tissue fibers. Microtubules are the largest and are composed of the pro-
Muscle tissue is contractile tissue. It can be classified as striated tein tubulin. Other fibers may be tissue specific: keratin fibers in
or smooth and may be under voluntary or involuntary control. epithelium, microfilaments of actin and myosin fibers in the sar-
Striated muscle has a regular pattern of light and dark bands coplasm of muscles, and neurofilaments in neurons. The cyto-
and is subdivided into skeletal and cardiac muscle. Skeletal skeleton is a three-dimensional scaffolding within the cytoplasm
muscle is under voluntary control, whereas cardiac muscle is that gives the cell structure and support and provides intra-
controlled involuntarily. The structure of skeletal muscle and cellular transport. The nucleus, the control center for the cell,
the mechanism of its contraction are discussed in Chapter 11. directs cellular function and contains most of the genetic mate-
The smooth muscle fiber is an elongated, slender cell with a rial within its deoxyribonucleic acid (DNA), which is organized
single centrally located nucleus. This tissue is under the invol- into chromosomes. The genes within the chromosomes are the
untary control of the autonomic nervous system. genome. Ribosomes, granules of ribonucleic acid and proteins
within the cytoplasm, manufacture proteins as directed by the
Nerve Tissue cellular DNA. The endoplasmic reticulum within the cytoplasm
Nerve tissue encompasses two types of cells: neurons, which provides sites for protein and lipid synthesis. Smooth endoplas-
are specialized cells that react to a stimulus and conduct a nerve mic reticulum does not have embedded ribosomes. It is involved
impulse, and neuroglia, which are cells that provide structure and in steroid and lipid synthesis. Rough endoplasmic reticulum
metabolic support to the neurons. The neuron cell body, called houses ribosomes and is involved in producing proteins. The
the soma, has several cytoplasmic projections. The projections Golgi apparatus modifies and packages proteins. Mitochondria,
that conduct impulses to the cell body are dendrites, and the pro- the powerhouse of the cell, produce the cell’s supply of energy
jection that conducts impulses away from the cell body is an axon. in the form of adenosine triphosphate (ATP). The inner wall of
A nerve impulse, in the form of an action potential, passes the double-walled mitochondria is folded into cisternae. This is
between nerves at a specialized junction, a synapse. As the where biochemical processes occur that result in the production
action potential reaches the presynaptic membrane of the first of ATP. Lysosomes, intracellular digestive systems containing
axon, a neurotransmitter is released into the synaptic gap, trig- powerful enzymes, take up bacteria or old organelles and break
gering an excitatory or an inhibitory response in the postsynap- them down into component molecules that are reused or reab-
tic membrane of the second neuron. sorbed into the cytoplasm and transported out of the cell.
Neuroglia in the central nervous system include oligodendro- Fluid and solute transport across a cell membrane can occur
cytes, astrocytes, and microglial cells. Schwann cells are the only passively either by diffusion down a concentration gradient or by
neuroglial cell in the peripheral nervous system. Cytoplasmic facilitated diffusion using membrane transport proteins (Fig. 1.8).
extensions of Schwann cells in the peripheral nervous system Molecules can be transported against the concentration gradient
encircle nerve fibers to form a myelin sheath, and oligodendro- with the use of active transport, which requires energy. Diffusion
cytes do the same in the central nervous system (including form- occurs when molecules pass from a higher to a lower concentra-
ing the myelin for the optic nerve). Nerve fibers thus are either tion and no energy is expended. Facilitated diffusion may occur
 CHAPTER 1  Introduction to the Visual System 7

Extracellular space

Glycoprotein Glycolipid
Outer
leaflet

Cholesterol Inner
leaflet
Fatty acid
tails Integral
Peripheral protein
Channel
protein

Polar head

Cytoplasm

Fig. 1.7  Model of the cell membrane. (From Gartner LP, Hiatt JL. Color Textbook of Histology.
3rd ed. Philadelphia: Saunders; 2007, p 16.)

through channel proteins or carrier proteins. Channel proteins cellular metabolism and are regulated by signals from either
within the cell membrane create water-filled passages linking the inside or outside the cell. Integrins are membrane-spanning
intracellular and extracellular spaces. These channels facilitate proteins that can carry information from the extracellular
ion movement across the lipid bilayer and move ions without the matrix into the cell and activate intracellular enzymes that
expenditure of energy. The channels control entrance into the cell then influence cellular processes. Energy for metabolic pro-
using gates. Voltage-gated channels open with depolarization. cesses is supplied by ATP molecules, produced either through
Ligand-gated channels open when a signaling molecule, such as aerobic or anaerobic metabolism. Aerobic metabolism is more
a neurotransmitter or a nucleotide like cyclic guanosine mono- efficient, with 36 to 38 molecules of ATP produced per mol-
phosphate, binds to the channel. Mechanical-gated channels open ecule of glucose. Anaerobic glycolysis yields two ATP per
with physical contact like cilia deformation. Some channels are molecule.
not gated, such as potassium (K+) channels or aquaporins, and are
always open. Transport across a cell membrane using carrier pro-
teins requires internal binding sites for the ion or molecule being
INTERCELLULAR JUNCTIONS
transferred. The carrier proteins never form a direct connection Intercellular junctions join epithelial cells to one another
between the intracellular and extracellular environments. This and to adjacent tissue. There are three main types of junc-
method is slower and selective but can carry larger molecules. tions. Tight junctions, which form fused connections between
Molecules, such as glucose and amino acids, are moved in this membranes of adjoining cells, include zonula occludens and
way. Carrier proteins can function passively (facilitated diffusion) macula occludens. Zonula adherens, macula adherens (des-
or with the use of energy (active transport). The most well-known mosomes), and hemidesmosomes form anchoring junctions
active transport pump is the Na+/K+ ATPase pump. Here, trans- between adjacent cells or between the cell and the basal lamina.
porters and cotransporters move substances against the concen- Gap junctions allow communication between adjacent cells
tration gradient and need a steady supply of ATP. Transporting by permitting passage of ions and small molecules between
epithelia are polarized and the apical and basal membranes have cells. Physical changes, such as pressure and biochemical or
differing properties. Both often contain ion channels; however, pharmaceutical factors, can modulate junctions and alter the
the Na+/K+ ATPase pumps are generally located in the basolateral junctional proteins. This allows changes in the extracellular
membranes. Aquaporins are bidirectional channels composed environment to be relayed to the interior cell and may affect
of major intrinsic proteins that specifically allow water passage intracellular processes.
but may not allow other materials to pass through the channel. With tight (occluding) junctions, the outer leaflet of the cell
Aquaporins are numerous in ocular tissues, including the cornea, membrane of one cell comes into direct contact with its neigh-
lens, ciliary body epithelia, and retina. bor. Ridgelike elevations on the surface of the cell membrane
Cellular metabolic functions are complex activities that fuse with complementary ridges on the surface of a neighbor-
maintain the viability of the cell. Amino acids, carbohydrates, ing cell. As the paired strands meet, the neighboring cell mem-
and lipids are used as building blocks in the construction of branes are fused. The fibers of tight junctions are connected to
cellular components or are broken down as a source of energy. the cytoskeleton within the cell. This forms an impermeable
A myriad of biochemical pathways and processes function in barrier that prevents passage of unwanted material between
8 CHAPTER 1  Introduction to the Visual System

Passive Transport
Extracellular space

Uniport Plasma
membrane

Simple diffusion Ion channel-mediated Carrier-mediated

of lipids diffusion diffusion

Facilitated diffusion
Cytoplasm
A
Active Transport

Extracellular space

Symport Antiport

Cytoplasm Coupled transport

B
Fig. 1.8 Types of transport. A, Passive transport that does not require the input of energy.
B, Active transport is an energy requiring mechanism. (From Gartner LP, Hiatt JL. Color Textbook
of Histology. 3rd ed. Philadelphia: Saunders; 2007, p 18.)

adjacent cells. Zonula occludens forms a belt-like zone of tight allows substances to pass between adjacent cells despite
junctions around the entire apical portion of the cell, joining it ­relatively firm adhesions. Adjacent to the adhering junctions
with each of the adjacent cells (Fig. 1.9). In these zones, row on are fine microfilaments that extend from a plaque just inside
row of intertwining ridges effectively occlude the intercellular the membrane to filaments of the cytoskeleton, contributing
space. A substance cannot pass through a sheet of epithelium to cell stability. In general, zonula adherens encircles the
whose cells are joined by zonula occludens by passing between entire cell just basal to the zonula occludens which lies near-
the cells. Instead the substance must pass through the cell. In est the cell apex (see Fig. 1.9B). Macula adherens (desmo-
stratified epithelia, where the surface layer is constantly being some) is a strong, spotlike attachment between cells (see Fig.
sloughed and replaced from below, zonula occludens, if present, 1.9A). A dense disc or plaque is present within the cytoplasm
will be located in the surface layer. The components of the tight adjacent to the plasma membrane at the site of the adher-
junction are found in increasing numbers as a cell moves from ence. Hairpin loops of cytoplasmic filaments called tonofila-
its origin in the basal layer until, finally, when the cell reaches ments extend from the disc into the cytoplasm and link to
the surface, its occluding junction is complete. The complex keratin filaments in the cytoskeleton, contributing to cell sta-
formed by the junctional proteins in the zonula occludens aids bility. Other filaments, transmembrane linkers, or cadherins
in forming the blood-retinal and blood-aqueous barrier. The extend from the plaque across the intercellular space, hold-
tight junction can be affected in some diseases, causing dys- ing the cell membranes together and forming a strong bond.
function of the barrier function. A macula occludens junction The intercellular space contains an acid-rich mucoprotein
has a rounded shape. that acts as a strong adhesive.
Zonula adherens and macula adherens are anchoring Hemidesmosomes provide a strong connection between
junctions that bind cells together. The adjacent plasma mem- the cell and its basement membrane and underlying connec-
branes are separated, leaving a narrow intercellular space tive tissue. They contain similar components to desmosomes.
that contains a glycoprotein material. This arrangement The protein complex extends through the cell membrane to
 CHAPTER 1  Introduction to the Visual System 9

ZO
ZO ZA

ZA

DESM
B

Connexin

MO
Connexon
HEMI-DESM

Cell 1 Cell 2

BM “Gap” between cells (~2nm)

A C
Fig. 1.9 Intercellular junctional complexes. A, The lateral cell membranes of adjacent cells.
Zonula occludens joins cells with no intercellular space present. Zonula adherens joins cells without
fusing the membranes. Macula adherens (desmosome) forms strong, spot-like junctions with fibers
extending into the cytoplasm. Hemidesmosomes form strong junctions that join the basal aspect of
the cell to its basement membrane. B, Zonula occludens and zonula adherens generally lie adjacent
to one another at the apex of the cell. C, Gap junctions joining two cells. Six proteins (connexins)
surround the central channel (connexon). BM, Basal membrane; DESM, desmosome; HEMI-DESM,
hemidesmosomes; MO, macula occludens; ZA, zonula adherens; ZO, zonula occludens.

attach to keratin in the basement membrane. Bundles of fila- with connexins of a neighboring cell forming a channel called
ments join the intracellular plaque to the underlying connec- a connexon (see Fig. 1.9C). These narrow channels allow rapid
tive tissue matrix, often attaching to a plaque embedded in the cell-to-cell communication, that is, passage of small molecules
connective tissue. and ions from one cell to another. A group of cells with such
Gap junctions are formed by a group of (usually six) pro- connections act like a syncytium, that is, a single cell with mul-
teins, called connexins, that span the cell membrane and unite tiple nuclei.