nner mitochondrial membrane: The inner mitochond- rial membrane is very rich in

proteins and the ratio of lipid to proteins is only 0.27:1 by weight. It contains high
proportion of the phospholipid cardiolipin. In contrast to outer membrane, the inner
membrane is virtually impermeable to polar and ionic substances. These sub-
stances enter the mitochondrion only through the mediation of specific transport
proteins.

• Cristae: The inner mitochondrial membrane is highly folded. The tightly packed
inward folds are called “cristae”.

Functional changes: It is now known that mitochondria undergo dramatic changes

when they switch over from resting state to a respiring state. In the respiring
state, the inner membrane is not folded into cristae, rather it seems to shrink
leaving a much more voluminous inter membrane space.

(c) Intermembrane space: The space between the outer and inner membranes is
known as the intermembrane space. Since the outer membrane is freely permeable
to small molecules, the intermembrane space has about the same ionic
composition as the cytosol.

(d) Mitochondrial matrix: The region enclosed by the inner membrane is known as
the mitochondrial matrix.

Composition of matrix: The enzymes responsible for citric acid cycle and fatty
acid oxidation are located in the matrix. The matrix also contains several strands
of circular DNA, ribosomes and enzymes required for the biosynthesis of the
proteins coded in the mitochondrial genome. The mitochondrion is not,
however, genetically autonomous, and the genes encoding most mitochondrial
proteins are present in nuclear DNA.

Functions

• Many enzymes associated with carbohydrates, fatty acids and nitrogen

metabolism are located within the mitochondrion. Enzymes of electron transport
and oxidative phosphorylation are also located in different areas of this cell
organelle.

Table 1.2 gives the names of some of the important

enzymes and their location.
 • The mitochondrion is specialised for the rapid

oxidation of NADH (reduced NAD) and FAD. H2 (reduced FAD) produced in

the reactions of glycolysis, the citric acid cycle and the oxidation of fatty acids.
The energy produced is trapped and stored as ATP, for future use of energy in the
body.

3. Endoplasmic reticulum (ER): Eukaryotic cells are characterised by several

membrane complexes that are interconnected by separate organelles. These
organelles are involved in protein synthesis, transport, modification, storage and
secretion.

Varying in shape, size and amount, the endoplasmic reticulum (ER) extends
from the cell membrane, coats the nucleus, surrounds the mitochondria and
appears to connect directly to the Golgi apparatus. These memb- ranes and the
aqueous channels they enclose are called cisternae.

CLINICAL ASPECT

A disease known as Luft’s disease involving mitochondrial energy transduction has been reported. Fu
by free radicals. Age related degenerative disorders such as Parkinson’s disease, cardiomyopat
mitochondrial damage.

Table 1.2: Location of some of the important enzymes in mitochondrion

Outer membrane Intermediate space Inner membrane Matrix

• Cytochrome b5 


• Cytochrome b5 reductase 


• Fatty acid CoA 
 synthetase 


• FA elongation system 


• Phospholipase A 


• Nucleoside diphosphokinase 


• Adenylate kinase

• Sulphite oxidase
 • Nucleoside diphospho-

kinase

• Cytochromes b, C1, C, aanda3

• NADH dehydrogenase
 • Succinate dehydrogenase

• Ubiquinone
• Electron-transferring flavo- proteins (ETF)

• Vector ATP synthase (F0F1)

• -OH-butyrate dehydrogenase

• Carnitine palmityl transferases

• All translocases

• Pyruvate dehydrogenase complex (PDH)

• Citrate synthase • Aconitase

• Isocitrate dehydrogenase (ICD)

• -oxoglutarate dehydrogenase

• Malate dehydrogenase • FA oxidation system

• Ornithine transcarbamoylase

• Carbamoyl phosphate synthetase I