Cell Physiology

Austin Busang Siziba

Office 88, West Wing
Physiology Unit
0778 124 173
Structure of a Generalized Cell

Figure 3.1
Cell physiology (pvt) Ltd

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Two ‘Categories’ of Organelles

• Membranous

• Non-Membranous

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Organization of cells
 Eukaryotic cells contain well defined cellular organelles such as:
Nucleus
Mitochondria
Endoplasmic reticulum
Golgi apparatus
Peroxisomes
lysosomes
MITOCHONDRIA
 In electron micrographs of cells, mitochondria appears as – rods, spheres or
filamentous bodies.
 Size: 0.5µm -1µm in diameter
up to 7µm in length.
 Features
 Mitochondria has got an inner membrane and an outer membrane. The
space between these two is called intermembranous space.
 Inner membrane convolutes into cristae and this increases its surface area.
 Both the membranes have different appearance and biochemical functions:
Biomedical importance
Inner membrane:
 It surrounds the matrix.
 It contains components of electron transport system.
 It is impermeable to most ions including H, Na, ATP, GTP, CTP etc and to large
molecules.
 For the transport of special carriers are present e.g. adenine nucleotide
carrier(ATP –ADP transport).
 Complex II i.e. Succinate dehydrogenase .
 Complex V i.e. ATP synthase complex.
Outer membrane:
It is permeable to most ions and molecules which can move from the
cytosol to intermembranous space.

Matrix:
 It is enclosed by the inner mitochondrial membrane.
 Contains enzymes of citric acid cycle.
 Enzymes of β-oxidation of fatty acids.
 Enzymes of amino acids oxidation.
 Some enzymes of urea and heme synthesis.
 NAD
 FAD
 ADP,Pi.
 Mitochondrial DNA.
 Mitochondrial cytochrome P450 system
a. Hydroxylation of cholesterol to steroid hormones (placenta, adrenal
cortex, ovaries and testes)
b. Bile acid synthesis (liver)
c. Vitamin D formation( kidney).
 Mitochondria plays a key role in aging-
Cytochrome c component of ETC (electron transport chain) plays a main
role in cell death and apoptosis.
 Mitochondria have a role in its own replication- they contain copies of
circular DNA called mitochondrial DNA, this DNA have information for 13
mitochondrial proteins and some RNAs. This is DNA inherited from
mothers.
 Most mitochondrial proteins are derived from genes in nuclear DNA.
 Mutation rate in mt DNA is 10 times more.

 Mitochondrial Diseases:
i. Fatal infantile mitochondrial myopathy and renal dysfunction
ii. MELAS(mitochondrial encephalopathy, lactic acidosis and stroke).
 Fatal infantile mitochondrial myopathy

• Its a severe autosomal recessive muscular dystrophy

with onset in the first weeks of life after a normal
neonatal period.
• Affected infants show rapidly progressive muscular
rigidity of the trunk and limbs associated with
increasing respiratory difficulty resulting in death
before age 3 years
i. Lebers hereditary optic neuropathy (mitochondrially transmitted from mother
to child)—lead to acute or subacute loss of central vision-affects males.
ii. Myoclonic ( brief involuntary twitch of a muscle or groups of muscles) epilepsy
iii. Ragged red fiber disease.

 Also implicated in:

Alzheimer’s disease, Parkinson’s , Cardiomyopathies and diabetes.
ENDOPLASMIC RETICULUM
 Cytoplasm of eukaryotic cells contain a network of interconnecting
membranes. This extensive structure is called endoplasmic reticulum.
 It consists of membranes with smooth appearance in some areas and rough
appearance in some areas-
Smooth endoplasmic reticulum and rough endoplasmic reticulum.
Biomedical importance
Rough Endoplasmic Reticulum
 These membranes enclose a lumen.
 In this lumen newly synthesized proteins are modified.
 Rough appearance is due to the presence of ribosomes attached on its
cytosolic side(outer side).
 These ribosomes are involved in the biosynthesis of proteins.
 These proteins are either incorporated into the membranes or into the
organelles.
 Special proteins are present that are called CHAPERONES. Theses proteins
play a role in proper folding of proteins.
 Protein glycosylation also occurs in ER i.e. the carbohydrates are attached
to the newly synthesized proteins.
Smooth Endoplasmic Reticulum
 Smooth endoplasmic reticulum is involved in lipid synthesis.
 Cholesterol synthesis
 Steroid hormones synthesis.
 Detoxification of endogenous and exogenous substances.
 The enzyme system involved in detoxification is called Microsomal Cytochrome
P450 monooxygenase system(xenobiotic metabolism).
 ER along with Golgi apparatus is involved in the synthesis of other
organelles –lysosomes & Peroxisomes.
 Elongation of fatty acids e.g. Palmitic acid 16 C- Stearic acid 18 C.
 Desaturation of fatty acids.
 Omega oxidation of fatty acids.
GOLGI APPARATUS
 Golgi complex is a network of flattened smooth membranous sacs-
cisternae and vesicles.
 These are responsible for the secretion of proteins from the
cells(hormones, plasma proteins, and digestive enzymes).
 It works in combination with ER.
 Enzymes in golgi complex transfer carbohydrate units to proteins to form of
glycoporoteins, this determines the ultimate destination of proteins.
 Golgi is the major site for the synthesis of new membrane, lysosomes and
peroxisomes.
 It plays two major roles in the membrane synthesis:
i. It is involved in the processing of oligosaccharide chains of the
membranes (all parts of the GA participates).
ii. It is involved in the sorting of various proteins prior to their
delivery(Trans Golgi network).
 Lysosomes
 These are responsible for the intracellular digestion of both intra and
extracellular substances.
 They have a single limiting membrane.
 They have an acidic pH- 5
 They have a group of enzymes called Hydrolases.
• Glycogen storage disease type II also called pompe disease is an autosomal
recessive metabolic disorder which damages muscle and nerve cells
throughout the body.
• It is caused by an accumulation of glycogen in the lysosome due to
deficiency of the lysosomal acid alpha glucosidase enzyme . can be treated
by enzyme replacement therapy (ERT)
 Biomedical importance
 The enzyme content varies in different tissues according to the requirement
of tissues or the metabolic activity of the tissue.
 Lysosomal membrane is impermeable and specific translocators are
required.
 Vesicles containing external material fuses with lysosomes, form primary
vesicles and then secondary vesicles or digestive vacoules.
 Lysosomes are also involved in autophagy.
 Products of lysosomal digestion are released and reutilised.
 Indigestible material accumulates in the vesicles called residual bodies and
their material is removed by exocytosis.
 Some residual bodies in non dividing cells contain a high amount of a
pigmented substance called Lipofuscin.
 Also called age pigment or wear –tear pigment.
 In some genetic disease individual lysosomal enzymes are missing and this
lead to the accumulation of that particular substance.
 Such lysosomes gets enlarged and they interfere the normal function of the
cell.
 Such diseases are called lysosomal storage diseases
 Most impt is I-cell disease.
 PEROXISOMES
 Called Peroxisomes because of their ability to produce or utilize
H2O2.
 They are small, oval or spherical in shape.
 They have a fine network of tubules in their matrix.
 About 50 enzymes have been identified.
 The number of enzymes fluctuates according to the function of
the cells.
 Biomedical importance
 Xenobiotics leads to the proliferation of Peroxisomes in the
liver.
 Have an important role in the breakdown of lipids,
particularly long chain fatty acids.
 Synthesis of glycerolipids.
 Synthesis of glycerol ether lipids.
 Synthesis of isoprenoids.
 Synthesis of bile.
 Diseases associated:
Most important disease is Zellweger Syndrome. There
is absence of functional peroxisomes. This leads to the
accumulation of long chain fatty acids in the brain,
decreased formation of plasmalogens, and defects of
bile acid formation.
 NUCLEUS
 The nucleus is the largest cellular organelle in animals. In
mammalian cells, the average diameter of the nucleus is
approximately 6 micrometers (μm), which occupies about
10% of the total cell volume. The viscous liquid within it is
called nucleoplasm, and is similar in composition to the
cytosol found outside the nucleus. It appears as a dense,
roughly spherical organelle.
 Eukaryotic cells contain a nucleus.
 It has got two membranes- nuclear envelope.
 Outer membrane is continuous with the membrane of
endoplasmic reticulum.
 Nuclear envelope has numerous pores. That permit
controlled movement of particles and molecules between
the nuclear matrix and cytoplasm.
 Nucleus has got a major sub compartment- nucleolus.
 Deoxyribonucleic acid (DNA) is located in the nucleus. It is the
repository of genetic information.
 Present as DNA- protein complex –Chromatin, which is organized into
chromosomes.
 A typical human cell contains 46 chromosomes.
 To pack it effectively it requires interaction with a large number of
proteins. These are called histones.
 They order the DNA into basic structural unit called Nucleosomes.
Nucleosomes are further arranged into more complex structures called
chromosomes
 THE NUCLEUS

• Site of DNA
• (In chromosomes)

• Has all machinery necessary to maintain,

copy, and transcribe DNA >>> focus for all
of the cellular pathways that regulate gene
expression and cell division

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 Nucleus

• Surrounded by a double membrane

• Outer membrane is studded with ribosomes and is continuous
with membranes of the rough ER (endoplasmic reticulum)
• The inner membrane is smooth and faces the intranuclear space
or nucleoplasm
• Space between these 2 is continuos with the lumen of the rough
ER

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 Nucleus

• The 2 nuclear membranes meet at nuclear pores

• Provides pathway between the cytoplasm to nuclear interior
• Passage of RNA transcripts
• Passage of signaling molecules that influence nuclear function

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 THE CYTOSKELETON

• This is a network of protein fibres extending throughout all

eukaryotic cells, used for support, transport and motility

• Determine cell shape and resilience

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Microtubules
Microfilaments
Thin Filaments
Thick filaments
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• Thin filaments (actin) and thick
filaments (myosin) are present in
almost every cell type

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