Professional Documents
Culture Documents
MIMI HERMAN
110467/2011
Overview jalur
metabolisme protein
Senyawa mengandung N
Karbon Nitrogen
• Oligopetida diserap sel usus halus, kemudian dengan bantuan enzim peptidase dipecah menjadi
asam amino, tripeptida dan dipeptida
• Selanjutnya dengan bantuan enzim peptidase tripeptida dan dipeptida dipecah menjadi asam
amino
• Asam amino yang terbentuk diserap oleh sel usus halus, selanjutnya masuk ke sistem tranport
darah
2. Reaksi Deaminasi
Contoh :
Glutamat + NAD+ + H2O α -KG+ NH3 + NADH
2. Reaksi Deaminasi
Contoh :
Glutamat + NAD+ + H2O α -KG+ NH3 + NADH
Hiperamonemia Tipe I
Defesiensi karbamoil fosfat sintase I
Hiperamonemia Tipe II
Defesiensi ornitin transkarboilase
Sitrulinemia
Defesiensi argininosuksinat sintase
Arginosuksinilkasiduria
Defesiensi argininosuksinase
Hiperargininemia
Defesiensi sintesis ureum.
All of the nitrogen in heme is derived from glycine and all of the carbons are
derived from succinate and glycine. Thus, the process by which heme is
synthesized is also called the succinate-glycine pathway. The first step in the
process is catalyzed by a pyridoxal phosphate-containing enzyme, -
aminolevulinic acid synthetase (ALA synthetase) The next step is the
synthesis of a substituted pyrrole compound, porphobilinogen from ALA
Metabolisme Heme lanjutan
Finally, the side chains are modified and the ring system is dehydrogenated
(Figure 21.30).
Note that uroporphyrinogen III is an asymmetric compound. It arises from
action of uroporphyrinogen I synthase and uroporphyrinogen III cosynthase.
If only the first enzyme is active, the symmetric compound,
uroporphyrinogen I is produced.