ENZYMES

AND
FUNCTIONS
OF MITOCHONDRIA
ENZYMES OF MITOCHONDRIA

• WHAT ARE ENZYMES?

Enzymes are macromolecular biological catalysts. Enzymes
accelerate or catalyze, chemical reactions.

• WHY ENZYMES ARE IMPORTANT?

Almost all metabolic processes in the cell need enzymes in
order to occur at rates fast enough to sustain life.
LOCATION

Enzymes are located at various places in mitochondria.

These are;
 Outer membrne
 Inner membrane
 Space between inner and outer membrane
 Mitochondrial matrix
 OUTER MEMBRANE

These enzymes are;

a) Monoamine oxidase
They belongs to the family of enzymes that catalyze the
oxidation of monoamines. The enzyme was originally
discovered by Mary Bernheim in the liver. They belong
to the protein family of flavin-containing amine
oxidoreductases.
b) NADH-CYTOCHROME C-REDUCTASE

Rotenone-insensitive NADH-
cytochrome c-reductase
• This enzyme belongs to
the family of
oxidoreductases,
specifically those acting on
diphenols and related
substances.
 c) KYNURENINE HYDROXYLASE
• Kynurenine production is increased
in Alzheimer's and cardiovascular
disease.
• Kynurenine and its further
breakdown products carry out
diverse biological functions. Some
cancers increase kynurenine
production, which increases tumor
growth.
• This enzyme helps in detoxification
of kynurenine.
 d) FATTY ACID CO-A LIGASE
• The long chain fatty acyl-CoA
ligase (or synthetase) is an
enzyme of the ligase family
that activates the breakdown
of complex fatty acids.

Fatty acid + CoA + ATP ⇌

Acyl-CoA + AMP + PPi.
OTHER FUNCTIONS

The outer membrane also contains enzymes involved in

such diverse activities as;
 Elongation of fatty acids
 Oxidation of epinephrine(neurotransmitter)
 Degradation of tryptophan
(Side effects include nausea, diarrhea, headache, dry
mouth, blurred vision, and nystagmus (involuntary eye
movements).
 INNER MEMBRANE

The inner mitochondrial membrane contains proteins with

five types of functions:
 Those that perform the redox reactions of oxidative
phosphorylation.
 ATP synthase, which generates ATP in the matrix.
 Specific transport proteins that regulate metabolite
passage into and out of the matrix.
 Protein import machinery.
 Mitochondrial fusion and fission protein.
 SPACE b/w INNER AND OUTER MEMBRANE

• Adenylate kinase (myokinase) is a phosphotransferase

enzyme that catalyzes the interconversion of adenine
nucleotides, and plays an important role in cellular energy
homeostasis.
• Nucleoside diphosphate kinases are enzymes required
for the synthesis of nucleoside triphosphates (NTP) other
than ATP. They provide NTPs for nucleic acid synthesis,
CTP for lipid synthesis, UTP for polysaccharide synthesis
and GTP for protein elongation.
 MITOCHONDRIAL MATRIX

• The Citric Acid Cycle is facilitated by;

Pyruvate dehydrogenase, Citrate synthatase, Aconitase,

Isocitrate dehydrogenase, α-ketoglutarate dehydrogenase,
Succinyl-CoA Synthetase, Fumarase, and Malate
dehydrogenase.
 MITOCHONDRIAL MATRIX

The Urea Cycle is facilitated by;

Carbamoyl phosphate synthetase I and Ornithine
transcarbamylase.
β-Oxidation uses;
Pyruvate carboxylase, Acyl-CoA dehydrogenase, and 𝛽-
Ketothiolase.
Amino acid production is facilitated by;
Transaminases
 FUNCTIONS
MITOCONDRIAL MATRIX
• The Citric Acid Cycle.
• Oxidative Phosphorylation.
• Urea Cycle.
• Transamination.
• Regulation.
• Protein Synthesis.
INTER-MEMBRANE SPACE

H+ ions build up in the

space between the
inner membrane and
outer membrane to
create a proton
potential that helps
power the ATP
formation. This space
is called the inter-
membrane space.
INNER MEMBRANE

The mitochondrial
inner membrane is
the site of the
electron transport
chain, an important
step in aerobic
respiration.