KuliahMetHbNIM10.ppt
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Transcript of KuliahMetHbNIM10.ppt
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METABOLISME HEMOGLOBIN
Eritrosit mengandung Hemoglobin yg penting untuk transportasi Oksigen.
HEME (SUATU PORPHYRIN)
Hemoglobin
GLOBIN (SUATU PROTEIN)
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SUKSINIL KoAGLISIN
PIRIDOXAL-pPORPHOBILINOGEN
UROPORPHIRINOGEN III
COPROPORPHIRINOGEN III
PROTOPORPHIRINOGEN III
PROTOPORPHIRIN I III
HEMEBILIRUBIN
UROBILIN
BILIVERDIN
B.INDIREKB.DIREK
UROBILINOGEN
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GLOBIN = PROTEIN
EKSPRESI GEN :•TRANSKRIPSI•* TRANSLASI
ASAM AMINO
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GLOBIN = PROTEIN
ASAM AMINO
•ENERGI•* SINTESA PROTEIN BARU
•* KH•* LIPID
DEGRADASI/KATABOLISME:*TRANSAMINASI
*DEAMINASI* SINTESA UREA
ENZIM PEMUTUS IKATAN PEPTIDA
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Enzim-enzim dalam proses biosintesa heme juga protein.
Sintesa protein mengikuti proses transkripsi dan translasi,dimana informasi kandungan asam amino dari protein ditentukan oleh kode genetika pada mRNA.
Kelainan genetik kemungkinan menyebabkan kelainan globin atau enzim-enzim yg mengkatalisir sintesa Heme.
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ACUTE INTERMITTEN PORPHYRIATanda-tanda penting:- Intermitten abdominal pain.
Penyebab nyeri abdomen: neurologik.- Seizure ,psychosis.- Hyponatremia.- Mutasi porphobilinogen deaminase gen THALASSEMIA-Globin dari Hb A disusun oleh 2 rantai- dan 2
rantai-.- Terjadi reduksi sintesa rantai globin.- Hypochromic microcytic anemia.- Thalassemia - : delesi gen.- Thalassemia- : point mutation.
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PORPHYRIN
Porphyria: sekelompok penyakit yang disebabkan kelainan pada jalur biosintesa berbagai porphyrin.
Porphyrin : senyawa siklis yg dibentuk oleh 4 cincin pyrrolemelalui jembatan —HC=.
Protein yg mengandung heme termasuk hemoprotein.
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