INTEGRATION OF AMINO ACID METABOLISM

BCH-102
Submitted to:Dr. JAVED ASAD Submitted by:-

Ghulam HASSAN Iqbal 07-arid-1711 DVM-2nd Semester

Conversion of AMINO ACIDS to Intermediates of TCA Cycle

Alpha-ketoglutarate
Glutamate Arg His Gln Pro Ala, Cys, Gly, Hyp, Ser, Thr Ile Ile, Leu, Trp Pyruvate

Citrate

Succinyl CoA

Met Val

Acetyl-CoA
Acetoacetyl CoA Leu, Lys, Phe Tyr, Trp

Oxalosuccinate
Aspartate Asn

Fumarate

Tyr Phe

Conversion of Amino acids to AlphaAlphaketoglutarate

NH2 O C CH2 CH2 H C COO H2O NH4

O +

C CH2 CH2

Pyr

Ala

(Transaminase)

(Glutaminase)

+ NH3

C COO-

+ NH3
O C CH2 CH2 C O

L-Glutamine

L-Glutamate

COO

Alpha ketoglutarate

Arginine
H2O Urea

(Arginase)

NH3 CH2 CH2 CH

+
OC O

CH2 NH3

L-Ornithine
Alpha-KG

Glutamine

L-Glutamate-r-seminaldehyde
NAD + (Glutamate semialdehyde NADH+H + dehydrogenase)

L-Glutamate

Conversion of Amino acid to oxalosuccinate

NH2 O C CH2 N C COONH2
(Asparaginase)

C CH2 C CO-

O
H2O Pyr Ala

+ NH3
(Transaminase)

L-Asparagine

L-Aspartate

C CH2

COO

Oxalosuccinate

Conversion of Amino Acids to Acetyl CoA

H3C CH OH + NH3 O CH C O

Glycine
(Threonine Aldolase)

CH3 CH O

L-Threonine

Acetaldehyde
H2O
NAD+

(Aldehyde Dehydrogenase)

NADH + H+

H3C C O

COA
COASH H2O

H3C C
ATP

(Acetate Thiokinase) ADP

Acetyl-CoA

Acetate

NH3 CH CH2 C O O O O

H3C C CH2 C

O-

L-Tyrosine
CoASH
(B-Ketothiolase)

H3C
C O

CoA

Acetyl-CoA

Conversion of Amino Acids to Intermediates of Glycolysis

H HO CH2 C NH3 COOH2O

H2C

C
+ NH3

COO-

Serine

Aminoacrylate

O CH2 C COO-

Pyruvate

Conversion of Alpha-ketoacid to AlphaAlphaAlphaAmino Acid

COO HC

COO-

+ NH3

Alpha ketoacid

Alpha ketoacid

C
(Aminotransferase)

CH2OH

Serine

CH2OH

3-Hydroxy pyruvate
(D-Glycerate Dehydrogenase) NADH+H + NADH

COOHC OH
(Glycerate kinase)

COO-

HC

ON

ATP

CH2

PO3

ADP

CH2OH

3-Phosphoglycerate

D-Glycerate

Conversion of Acetyl CoA to Fats

Acetyl CoA Acetoacetyl CoA
H2O Acetyl CoA

Ketone bodies

HMG CoA

(HMG-CoA Reductase)

2NADPH+2H+ + 2NADP

Mevalonic acid+CoA

Cholesterol

Reactions & Intermediates of Urea Biosynthesis

Urea H2O (Arginase)

L-Ornithine

L-Arginine

(Ornithine Transcarbamoylase)

(Arginosuccinase)

Fumarate

L-Citrulline
(Arginosuccinic Acid Synthase)

Arginosuccinate

AMP+Mg-PPi

L-Aspartate
Mg-ATP

Arginase Deficiency
Hyperargininimia. Abnomalities

in development and functioning of

CNS. Arginine accumulated and more excreted.

Ornithine Transcarbamoylase Deficiency

common deficiency in urea cycle. X related deficiency more common in males. Mental retardation and death often occurs because of excess NH3.
Most

Formation of Catecholamines and Melanin

Tyrosine DOPA
(Decarboxylation)

Dopamine

(Hydroxylation)

(Methylation)

Epinephrine

Norepinephrine

Tyrosine is required for the synthesis of melanin.

Albinism
    

It is the deficiency of Tyrosinase. Sensitive to light. No pigments. Increasing incidence of skin cancer. Photophobia.

References
 Devlin.T,2002,Textbook

of Biochemistry with

clinical correlations. WileyWiley-Lyss New York.CH Amino acid metabolism.  www.rpi.edu/dept/bcbp/molbiochem/MBWeb/ mb2/part1/23mb2/part1/23-aacarb.ppt.