Deamination of Amino Acids - The Urea Cycle

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DEAMINATION OF AMINO ACIDS - THE UREA CYCLE

AMINO ACIDS

AMINO ACIDS

+
RCH(NH3)COO-

RCH(NH3+)COO-

AMINO ACID I

AMINO ACID II

MAINLY LEUCINE, ISOLEUCINE, VALINE


-

OOCCH2CH2COCOO-

2-Oxoglutarate
2.6.1.

-OOCCH

CH

CH

R.COCOO

(NH3+)

2.6.1.

2-Oxo acid

COO-

-OOCCH2CH2CH

GLYCOLYSIS
Glucose

Glutamate

GLUCOSE
ALANINE
CYCLE

(NH3+)

(NH3+)
GLUTAMATE

-OOCCH

CH

CH

Glutamate
+
NAD(P)
1.4.1.3

COO-

N-Acetylglutamate

2-Oxo acid

2-Oxoglutarate

COO-

HO

UREA CYCLE

6.3.4.16

REACTANTS

PRODUCTS

HCO3-

NH4+

3.5.1.2

Glutamate

2ATP

2ADP

Oxaloacetate

2-Oxoglutarate

H2N-CO-OP

Carbamoyl-

Glutamine

MITOCHONDRIAL INNER MEMBRANE


MITOCHONDRIAL MATRIX

Ornithine

(NH +)
GLUTAMATE3

-OOCCH2CH2CH

Gluconeogenesis

NH +

H3N[CH2]3CH(NH3+)COO-

AMINO ACID II

Catabolism etc.

OXIDATIVE
DEAMINATION

NAD(P)H

CYTOSOL

2-Oxoglutarate

esi

Glu

one gen
o

COO-

CELL MEMBRANE

LIVER

COO-

AMINO ACID I

Pyruvate

(NH3+)

Glutamine

SKELETAL & HEART MUSCLE, KIDNEY

Alanine

2.6.1.2

Glucose

ADP+Pi
H2NOCCH2CH2 CH

COO-

Alanine

CH CH
3

(NH3+)

6.3.1.2

Transport to Liver

2.6.1.2

CH CH

COO-

NH4++ ATP

Catabolism and/or

Pyruvate

MUSCLE

(NH3+)

Glutamate

Glutamate

2.6.1.1.

2.1.3.3

Ornithine

H2N-C-

U
R
E
A
C
Y
C
L
E

NO.

Citrulline

HN[CH2]3CH(NH3+)COO-

OOCCH CHCOO
2

(NH3+ )

Citrulline

Gluconeogenesis

Aspartate
- OOCCH

CHCOO-

6.3. 4.5

ATP

NH
H+2N=C- HN[CH2]3CH(NH

1.5 NADPH

AMP
3

PP

)COO-

4.3.2.1

OOCCH(OH)CH COO
2

NADH+H+

Malate

Fumarate

HN[CH2]3CH(NH3+)COO-

H O
2

Arginine

UREA CYCLE

OOCCH=CHCOO

NH2
+
H2N=C-

OOCCOCH COO

Oxaloacetate

Argininosuccinate

1.14.13.39

+
1.5 NADP

1.5O2

Aspartate

MITOCHONDRIAL INNER MEMBRANE

NAD+

3.5.3.10

H2N-CO-NH2

H3N[CH2]3CH(NH3+)COO-

UREA

Ornithine
CELL MEMBRANE

NH4+ + HCO3- + -OOC.CH2CH(NH3+)COO- + 3ATP

UREA CYCLE EQUATION

H2N.CO.NH2 + -OOCCH=CHCOO- + 2ADP + 2Pi + AMP +PPi


UREA

Aspartate

Urea

Glutamine
3.5.1.2

NH4+

Fumarate

Glutamate
1.4.1.3

NH4+

ENZYMES
1.1.1.37
Malate dehydrogenase
1.14.13.39 Nitric-oxide synthase
1.4.1.3
Glutamate dehydrogenase (NAD(P)
2.1.3.3
Ornithine carbamoyltransferase
2.6.1.Amino acid aminotransferase
2.6.1.1
Aspartate aminotransferase

2.6.1.2
3.5.1.2
3.5.3.10

Alanine aminotransferase
Glutaminase
Arginase

6.3.1.2

Glutamate-ammonia ligase
(Glutamine synthase)

6.3.4.5

Argininosuccinate synthase

4.2.1.2
4.3.2.1

Fumarate hydratase
Argininosuccinate lyase

6.3.4.16

Carbamoyl-phosphate synthase

2001

IUBM B

(ammonia)

2-Oxoglutarate

Glucose

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