Bg hoa sinh 2 phan 2 0207

CHlJdNG CHUYEN HOA HEMOGLOBIN MUC TIEU Trinh bay va giai thich dtfdc tien trinh va cd chd cua qua trinh sinh tdng hdp hemoglobin Trinh bay va giai thich dtfdc tien trinh va cd che ciia qua trinh thoai hda hemoglobin Neu va giai thich dtfdc nhtfng benh ly gay rd'i loan chuyen hda hemoglobin SINH TONG H0P HEMOGLOBIN 1.1 Sinh tdng hcfp hem Hem cd cau triic gdm nhan protoporphyrin III ket hdp vdi Fe"+ Stf tdng hdp hem xay hau het te bao ngoai trtf hong cau trtfdng vi khong cd ty the Khoang 85% stftdng hdp hem xay d te bao tien hong cau xtfdng 1.1.1 Tong hcfp nhan protoporphyrin III Hai nguyen lieu khdi dau cho qua trinh tdng hdp nhan porphyrin ttf tao hem la succinyl CoA va glycin Succinyl CoA la mot cha't trung gian ciia chu trinh acid citric, glycin la mot acid amin Qua trinh tdng hdp nhan protoporphyrin qua cac giai doan sau: 1.1.1.1 Tao acid - amino levulinic (ALA) Stf tdng hdp ALA xay d ty the, ndi ma succinyl CoA dtfdc tao cac phan tfng ciia chu trinh acid citric Glycin dtfdc hoat hda bdi pyridoxal phosphat (vitamin B6) Succinyl CoA ket hdp vdi glycin, loai CoA tao acid a - amino -P - ceto adipic Chat nhanh chdng bi khtf carboxyl de tao acid -amino levulinic (ALA) vdi stf xiic tac ciia enzym ALA synthetase vdi coenzym la pyridoxal phosphat: COOH I CH9 COOH I CH9 Succinyl CoA I CH I _ I I CH2 I CH2 I CO-SCoA HSCoA CO Glycin + CH2-NH2 i00u COOH I CHo ALA synthetase pyridoxal phosphat fO *" CH-NH2 J^n "; (COOjH T^n*" UU2 CH2-NH2 Ac[d a.amlno levulinic (ALA) Acid a-amino p-cetoadipic 1.1.1.2 Tao porphobilinogen ALA dtfdc tao se di chuyen den bao ttfdng Tai day, hai phan ttf ALA ngtfng tu vdi (loai phan ttf H20) va dong vdng pyrol tao porphobilinogen vdi stf xiic tac ciia enzym ALA dehydratase (porphobilinogen synthase), enzym bi tfc che bdi chi kim loai (Pb) 102 COOH COOH I COOH I CH2 CH2 CHP C A NH2 - CH2 C Ql_j OH N COOH CK ^2Ha° I H2 O COOH CH /\LA dehydratase / NH^CH^ ^N/VH NH2-CH2 N H Porphobilinogen 1.1.1.3 Tao uroporphyrinogen Bon phan ttf porphobilinogen ket hdp vdi nhau, loai 3NH3 tao mot tetrapyrol mach thing va chat lai tidp tuc loai NH3 va dong vong, cac nhan pyrol ndi vdi blng ciu ndi CH2 cd the tao uroporphyrinogen I (enzym xiic tac ia uroporphyrinogen synthetase) va uroporphyrinogen HI (enzym xuc tac la uroporphyrinogen cosynthetase) Hai chat chi khac ve vi tri ciia 2nhdm the d nhan pyrol TV Binh thtfdng, dong phan dtfdc tao hau nhtf deu la uroporphyrinogen III Khi cd rdi loan chuyen hda porphyrin mdi tao uroporphyrinogen I •PA, -P A 3NH3 H,N-CH CH2/Vs^AcH2Ax^\cH2 H.N-CH, N H \ ,p N uroporphyrinogenase IV ^NH A\ _ hn( II N A p/ N H A /" A N IV )nh hn( II N \ Uroporphyrinogen p'\ Uroporphyrinogen 103 1.1.1.4 Tao coproporphyrinogen III , , ~- Uroporphyrinogen III chuvdn coproporphyrinogen III nhd stf khtf carboxyl cua tot ca cac gdc acetat (A) d mach nhanh dd chuydn cac gdc metyl (M) vdi stf xiic tac cua enzym uroporphyrinogen decarboxylase ,M IV >NH HN , , ,.x va 17 Ndng dp dtfdng Itfdng ciia cac cation va anion tronghuye't ttfdng 18 Chtfc nang ciia bach clu la 19 LDH co isoenzym 20.Ty cua cac lipoprotein phu thudc vao phin 209 DAP AN CHU'O'NG 1: CHUYEN HOA GLUCID l.a 3.b 5.a,d 7.a,c 9.b ll.a,c 2.c 4.a,d 6.a,b 8.a,b,d 10.a,b,c,d 12.c CHl/CfNG 2: CHUYEN HOA LIPID l.b 3.b 5.d 7.a,c,d 9.a,b,c 2.a 4.c 6.a,b,c,d 8.a,b,c,d 10.a,c CHlJCfNG 3: CHUYEN HOA PROTEIN VA ACID AMIN - SINH TONG HCfP PROTEIN 26.S l.d 6.b ll.D 16.S 21.D 2.b 7.a 12.S 17.D 22.D 3.c 8.a 12.S 18.S 23.D 28.D 4.a 9.a 14.D 19.S 24.D 29.D 5.b lO.b 15.D 20.S 25.S 30.S 27.S - CHl/CfNG 4: CHUYEN HOA HEMOGLOBIN CHlJCfNG 5: CHUYEN HOA ACID NUCLEIC l.a,b,c,d 3.b,c,d 5.b,d 7.c 9.a 2.a,b,d 4.a,b,c,d 6.b 8.d lO.d CHLfCfNG 6: LIEN QUAN VA DIEU HOA CHUYEN HOA l.D 3.S 5.S 7.D 9.S 2.D 4.S 6.D 8.S 10.S 210 CHUCfNG 7: SU TRAO DOI NUCfC VA CAC CHAT DIEN GIAI l.b 3.d 5.c 7.a,c 9.a,b,c,d 2.d 4.a 6.a 8.a,b,c,d 10.c,d CHUCfNG 8: THANG BANG ACID - BASE l.a 3.d 5.c 2.b 4.a 6.a,b,c,d CHUCfNG 9: HOA SINH THAN VA NUCfC TIEU l.d 5.d 9.d 13.b 17.a,b,c,d 2.b 6.d lO.c 14.a,b,d 3.c 7.c ll.d 15.a,c,d 18.b,c,d 19.a,b,c,d 4.b 8.a 12.d 16.a,b,c,d 20.a,b,c CHUCfNG 10: HOA SINH GAN l.b 5.b 9.b 13.b 17.a,d 2.d 6.b lO.d 14.b,c 18.b,d 3.d 7.c ll.c 15.a,b,d 19.b,c,d 4.c 8.c 12.c 16.a,d 20.a,d CHUCfNG 11: HOA SINH MAU l.b 6.D ll.D 2.d 7.D 12.S 3.c 8.S 13.D 4.a 9.S 14.D 5.c 10.D 15.S 211 TAI LIEU THAM KHAO Tieng Viet Bd mdn Hda sinh-Trtfdng Dai hoc Y Ndi Hda sinh, NXB Y hoc Ha Ndi, 2001 Bd mdn Hda sinh - Dai hoc Y dtfdc TPHCM, Hda sinh y hoc NXB Y hoc TP HCM 2003 3.Nguyen xuan Thang, Hda sinh dtfdc ly phan ttf, NXB Khoa hoc ky thuat, 2003 4.Pham thi Tran Chau, Trln thi Ang, Hda sinh hoc, NXB Giao due 2004 Bo Y Te, Vu Khoa hoc va Dao tao, Hda sinh hoc, NXB Y hoc Ha Ndi, 2005 Bd Y Te, Sinh hoc phan ttf, NXB Giao due, 2007 Tieng ntftfc ngoai l.F Percheron, R Perles, M.J Foglietti, Biochimie structurale et metabolique, Tome et 2, Masson, 1992 • * P Valdiguie, Biochimie climque, EM Inter, 1993 Victor L Davison, Donald b Sittman, Biochemistry, * edition, Harwal Publishing, 1994 P.N Campbell Biochemistry Illustrated, 4* edition, Churchill hvingston, 2000 B.D Haames, N.M Hooper & J.D Houghton, L'Essentiel en Biochimie, Berti edition, 2000 Peter N Campbell; Anthony D Smith Biochemistry Illustrated.4th edition, Harwal publishing, 2001 R.K Murray, D.K Granner, V.W Rodwell-Harper's Illustrated Biochemistry 27th edition-Lange Medical Books/Mcgraw-Hill, 2006 C.A Burtis , E.R Ashwood, D.e Bruns - Tietz Textbook of Clinical Chemistry and Molecular Diagnostics 4th edition- Elsevier saunders, 2006 IMIHOCVOTRIMmSTOflN thUviIn 212 *