TCA

What is it for?

Provides majority of ATP

for energy
Gluconeogenesis from
skeletons of AA
Building blocks for AA &
Heme (succinyl CoA)

In ALL cells with

mitochondria
Mitochondrial matrix

Where does it
occur?

EXCEPT: succinate
dehydrogenase (inner
membrane)
Acetyl CoA

What is/are
the
substrate(s)?
What is/are
the end
product(s)?

GLYCOLYSIS

Cytoplasm (ALL cells)

Glucose

Pyruvate or Lactate

CO2
GTP
NADH
FADH2

GLUCONEOGENESIS

Breakdown of glucose into

3-C compounds to provide
energy

GLYCOGENESIS

Production of glucose
from the following
intermediates:
1) Intermediates of :
glycolysis & the TCA
2) Glycerol: from
Triacylglycerols
3) Lactate: through Cori
Cycle
4) C skeletons (
ketoacids) of
glucogenic amino
acids

WELL-FED

Liver (90%)
Kidney (10%)
Mitochondria
Cytoplasm

Liver
Muscle
Cytosol

Pyruvate

Glucose

Synthesis of new
glycogen molecules
from -D-glucose

1) UDP-glucose
2) ATP & UTP
3) Glycogenin: core,
primer protein
Glycogen

Depending on the
availability of
mictochondria & O2

GLYCOGENOLYSIS

PPP

FASTING

Production of important
intermediates:
Shortening of glycogen NADPH provides
chains to produce
electrons for:
molecules of a DFA & Steroid
glucose
biosynthesis
Reduction of
Glutathione
CYP450
WBC Respi burst
NO synthesis
Ribose-5-phosphate
Synthesis of
nucelotides
Metabolic use of 5-C
Liver
Cytoplasm
Muscle
Cytosol / Cytoplasm

Glycogen

G-6-P

Leaves about 4 glucose

No consumption or
residues before a
production of ATP
breanch point a
limit dextrin
Glucose-1-P
Ribose-5-P
Free glucose
F-6-P
Glyceraldehyde-3-P
Liver
NADPH
-

can release free

glucose to circulation

Muscle
-

Limited to G-6-P within

muscle only

Free glucose
-

Which step is
rate limiting?
Rate limiting
enzyme
Diseases

Isocitrate
ketoglutarate

F-6-P F-1,6-BP

F-1,6-BP F-6-P

Elongation of
glycogen
addition of (14)
bonds

Isocitrate dehydrogenase

PFK-1

F-1,6-Bisphosphotase

Glycogen synthase

Pyruvate dehydrogenase

Hypoglycemia

Glycogen storage

Produced during the

debranching process

Removal of glucose
Breaks (14) bonds

G-6-P 6Phosphogluconate

Glycogen
phosphorylase

G-6-P dehydrogenase
G6PD Deficiency

activity
Choronic alcoholism

FA synthesis
What is it for?

Where does it
occur?

Formation of Palmitate
(16:0)

Cytosol
Major: Liver, Lactating
mammary glands
Minor: Adipose tissue

What is/are the

substrate(s)?

1 Acetyl CoA
7 Malonyl CoA NADPH
ATP

What is/are the

end product(s)?

Palmitate ONLY

Which step is rate

limiting?
Rate limiting
enzyme

Acetyl CoA + ATP

Malonyl CoA
Acetyl CoA carboxylase

diseases

oxidation

Cholesterol synthesis

Gets Acetyl CoA fragments

from the ends of FAs
Acetyl CoA can enter the
citric acid cycle
Generates NADH & FADH2
that can enter the ETC

De novo synthesis of
cholesterol

Mitochondria of almost all

cells

Virtuall ALL cells

Exceptions:
Neurons
RBC
Testis
Kidney
Medulla
Palmitrate
NAD+
FAD
ATP
8 Acetyl CoA
7 FADH2
7 NADH
Fatty acyl CoA + Carnitine
Fatty Acyl carnitine +
CoA
Carnitine acyltransferase

Cytosol
SER

Chronic granulomatous
disease

Steroid hormone
synthesis
Cholesterol is precursor of
ALL steroid sex hormones
Glucocorticoids (Cortisol)
-without this
hypoglycemia in stressful
situations
Mineralocorticoids
(Aldosterone)
-fluid regulation
-without this
Hypervolemia
Sex hormones
(Testosterone &
Estradiol)
SER of:
Adrenal cortex
Ovaries & Testes
Placenta

Liver & Intestines

(majority)
Acetyl CoA
NADPH
ATP

Cholesterol
Pregnenolone (mother
hormone)

Lanosterol Cholesterol
HMG CoA Mevalonate

Cholesterol
Pregnenolone

HMG CoA reductase

Desmolase
Blocker:
Aminogluthetimide

Diseases

Fatty liver
Carnitine deficiency
MCAD deficiency
Jamaican vomiting
sickness
Refsums disease

Zellwegers syndrome
X-linked ALD