METABOLISME ASAM AMINO

LINGKUNGAN

ORGANISME

Biosintesis

Protein
Yang
dicerna

Protein
2

a
ASAM
AMINO
c

Degradasi n
(required)

b
c
Purin
Pirimidin
Porphyrin
Rangka
Karbon

Nitrogen
(ketogenic)
Urea
acetoacetate
acetyl CoA

(glucogenic)
digunakan
Untuk
Energi

pyruvat
-ketoglutarat
succinyl-CoA
Fumarate
Oksaloasetat

Amino Acid Requirements of Humans

-------------------------------------------------------------------Nutritionally Essential Nutritionally Nonessential
-------------------------------------------------------------------Argininea
Alanine
Histidine
Asparagine
Isoleucine
Aspartate
Leucine
Cysteine
Lysine
Glutamate
Methionine
Glutamine
Phenylalanine
Glycine
Threonine
Proline
Tryptophan
Serine
Valine
Tyrosine
--------------------------------------------------------------------a
Nutritionally semiessential. Synthesized at rates
inadequate to support growth of children.

KESEIMBANGAN
NITROGEN
Keseimbangan Nitrogen = nitrogen yg dicerna - nitrogen yg diekskresikan
(terutama sbg protein)

(terutama sbg urea)

Keseimbangan Nitrogen = 0 (nitrogen equilibrium)

sintesis protein = degradasi protein
Keseimbangan nitrogen Positive
sintesis protein > degradasi protein
Keseimbangan nitrogen Negativ
sintesis protein < degradasi protein

TRANSAMINASI

UREA CYCLE

mitochondria
cytosol

Fungsi : detoksifikasi ammonia

(mencegah hyperammonemia)

FATE OF THE CARBON SKELETONS

Rangka karbon digunakan untuk energi

Glucogenic: siklus TCA intermediates

atau pyruvate (gluconeogensis)

Ketogenic: acetyl CoA, acetoacetyl CoA,

atau acetoacetate

Metabolisme Purin
dan
Pyrimidin

Basa utama

Sumber masing2 atom pada cincin purin

CO2
Glycine

Aspartate

(amine)

N
8

2
10

N -Formyl-FH4

N
3

N
9

Glutamine (amide)

N10-Formyl-FH4

Kesimpulan & Regulasi

Ribose-5-phosphate
5-Phosphoribosyl-1-pyrophosphate (PRPP)

5-Phosphoribosylamine

IMP

Adenylosuccinate

XMP

AMP

GMP

Inhibisi Biosintesis Purin oleh Agen

Antitumor , 6-Mercaptopurine

1)

6-Mercaptopurine dirubah menjadi nukleotida

2)

Nukleotida tsb menghambat biosintesis pada

tahap 2, 12a, 12b, dan 13a.

Cytosine (C)
Thymine (T)

Uracil (U)

Basa utama

Sumber atom cincin pirimidin

4

Glutamine
3

Aspartate
6

CO2

N
1

Degradasi DNA dan RNA

Salvage Pathway for Purines

(~90%)

Lesch-Nyhan Syndrome

Degradasi Purin (~10%)

Allopurinol
Inhibisi xanthine oxidase

He
me

Struktur

N
Pyrrole

Porphyrias

hemoglobin

Fe
(digunakan
kembali

heme

globin

degradasi
(bilirubin)

Asam
amino
bebas

Heme
Biliverdin
Unconjugated bilirubin
Unconj.bilirubin/
albumin complex

Reticuloendothelial
system

Systemic circulation

Kidney
Unconj. bilirubin

Hepatocytes

Bilirubin
diglucuronide

urine

Bilirubin diglucuronide
Stercobilins

Urobilinogen

Large intestine

Bilirubin
Small intestine

HYPERBILIRUBINEMIA

-- elevated bilirubin in serum (above 1 mg/dL)

-- can be conjugated or unconjugated or both
depending on the situation
-- elevated bilirubin can diffuse into tissues,
making them appear yellow (jaundice)

HYPERBILIRUBINEMIA
Clinical Consequences:

-- Conjugated hyperbilirubinemia: benign

-- Unconjugated hyperbilirubinemia: benign at
concentrations < 25 mg/dL (albumin capacity)
-- At concentrations >25 mg/dL, unconjugated
bilirubin is free (uncomplexed) and can
enter the brain.
bilirubin encephalopathy (kernicterus)

Causes of JAUNDICE
1) Hemolytic anemia
-- destruction of erythrocytes
2) Hepatitis or cirrhosis
-- conjugation and excretion of bilirubin
3) Bile duct obstruction
-- conjugated bilirubin not delivered to intestine;
it backs up, spills over into the blood
4) Neonatal physiological jaundice
-- immature hepatic system of the newborn:
uptake, conjugation, excretion of bilirubin