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MM13__UR.AI

DEAMINATION OF AMINO ACIDS - THE UREA CYCLE

AMINO ACIDS

AMINO ACIDS

RCH (NH 3 ) COO -

+

RCH (NH 3 ) COO -

+

AMINO ACID I

MAINLY

LEUCINE, ISOLEUCINE, VALINE

AMINO ACID II

- OOCCH 2 CH 2 COCOO -

2-Oxoglutarate

2.6.1.

R.COCOO -

2.6.1.

- OOCCH 2 CH 2 CH (NH 3 + ) COO -

2-Oxo acid

- OOCCH 2 CH 2CH (NH 3 + ) COO -

Glutamate

Glutamate

Glutamate

NH 4 +

O

Catabolism and/or

+ATP

-

Pyruvate

Glucose

Transport to Liver

6.3.1.2

ADP+P i

MUSCLE

2.6.1.2

CH 3 CH (NH 3 + ) COO -

H 2 NOCCH 2 CH 2 CH (NH 3 + ) COO -

Glutamine

Alanine

SKELETAL & HEART MUSCLE, KIDNEY

GLUCOSE

ALANINE

CYCLE

CH 3 CH (NH 3 + ) COO -

CELL MEMBRANE

LIVER

Alanine

CYTOSOL

2.6.1.2

AMINO ACID I

2-Oxoglutarate

AMINO ACID II

Glutamine

Glucose

Pyruvate

- OOCCH 2 CH 2 CH (NH 3 + ) COO -

Catabolism etc.

- OOCCH 2 CH 2CH (NH 3 + ) C OO -

2-Oxo acid

GLUTAMATE

GLUTAMATE

Gluconeogenesis

o

MITOCHONDRIAL INNER MEMBRANE

Glutamate

MITOCHONDRIAL MATRIX

2-Oxoglutarate

NAD(P) +

H 2

1.4.1.3

OXIDATIVE

DEAMINATION

UREA CYCLE

REACTANTS PRODUCTS

NAD(P)H

3.5.1.2

N-Acetylglutamate

NH 4 +

HCO 3 -

2ATP

NH 4 +

6.3.4.16

Glutamate

2ADP

Oxaloacetate

H 3 N[CH 2 ] 3 CH(NH 3 + )COO -

Carbamoyl-P

H 2 N - CO -OP

P i

2.6.1.1.

Ornithine

2-Oxoglutarate

2.1.3.3

Citrulline

P i

Aspartate

Ornithine

MITOCHONDRIAL INNER MEMBRANE

O

H 2 N - C-

HN[CH 2 ] 3 CH(NH 3 + )COO -

- OOCCH CHCOO

2

(NH )

3

-

Citrulline

+

U

R

E

A

NO .

Aspartate

Gluconeogenesis

6.3. 4.5

ATP

- OOCC H CHCOO -

2

AMP

PP

1.5 NADPH

+

NH

Oxaloacetate

- OOCCOCH 2 COO -

H 2 N= C-

HN[CH 2 ] 3 CH(NH 3 + )COO -

i

1.14.13.39

Argininosuccinate

C

Y

C

L

E

1.5 NADP +

NADH+H +

4.3.2.1

- OOCCH=CHCOO -

- OOCCH(OH)CH 2 C OO -

Fumarate

Malate

NH 2

1.5O 2

+

H 2 N= C-

HN[CH 2 ] 3 CH(NH 3 + )COO -

H 2 O

NAD +

Arginine

UREA CYCLE

3.5.3.10

H 3 N[CH 2 ] 3 CH(NH 3 + )COO -

H 2 N- CO- NH 2

UREA

Ornithine

CELL MEMBRANE

NH 4 + + HCO 3 - + - OOC.CH 2 CH (NH 3 + ) COO - +3ATP H 2 N . CO . NH 2 + - OOCCH=CHCOO - + 2ADP + 2P i + AM P +PP i

UREA CYCLE EQUATION

Urea

Glutamine

3.5.1.2

+

Aspartate

UREA

Fumarate

NH 4

Glut a mate

1.4.1.3

ENZYMES

NH 4

+

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 Alanine aminotransferase

6.3.1.2 Glutamate-ammonia ligase

(Glutamine synthase)

6.3.4.5 Argininosuccinate synthase

2 -Oxoglutarate

3.5.1.2 Glutaminase

3.5.3.10 Arginase

4.2.1.2 Fumarate hydratase

4.3.2.1 Argininosuccinate lyase

6.3.4.16 Carbamoyl-phosphate synthase

Glucose

(ammonia)

c 2001 IUBM B

1.1.1.37 Malate dehydrogenase

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