14 : 616.155.16 . , , » - http://www.famous-scientists.ru , - . 65 , . -6. . , - [6]. , -6- , , ), , , [13]. , , , , 100 . [3; 4]. - , - , , - , - , , [9]. , . 90% , , . , - 2004 2007 . , - - [7; 10; 11; 12]. - - . , . 6,6 18 [1]. 100.000 , . 7, 2008 - 15 5-11- [14]. Beutler E. ( Hoefer MacroVue UV-25, Amersham Bioscience, USA) [2]. Thermal cycler Biocycler TC-S (Roche, USA) , [8; 17]. ( Codon 8 N. ( EPS3501XL, USA) Multiphor II, - c 3,5-9,5 ( USA) [15; 16]. [5]. ) 15, 16, 30, 31, - 40 IVS-1-110 M (G-A), 41 IVS-1-110 N, 49 IVS-2-1 M (G-A), 77 IVS-2-1 N, 46 IVS-1-6 M (T-S), 54 Codon 8 M (-AA), 55 9182 , 103 ( 56,60%, 46 25,27%, 33 18,13% ( - ) – - .1). 30 25 ,% 20 15 10 5 0 1 2 3 .1. 1. ; 2. ; 3. - 1(G-A); 5I - -IVS-1-5(G-S); 6I – +-IVS-1-6(T-S). IVS-1-110(G- ), IVS-2-1(G- ), IVS-16(T-S), IVS-1-5(G-S) , codon 8(- ) , ( . 1). + 65 : 110- I -IVS-1-110(G-A); 80 – -codon 8(- ); 1II 0 – + I 0 -IVS-2- 7, 2008 16 1. ,% + 30,77 16,92 13,85 6,15 3,08 -IVS-1-110(G- ) -codon8(-AA) 0 - IVS-2-1(G-A) + -IVS-1-5(G-S) + -IVS-1-6(T-S) 0 6,15 3,08 - 22( . 2). 13- 13,85 6,15 , 11- – , 25 ,% 20 15 10 5 0 1 2 3 4 5 . 2. 1. IVS-1-110(G-A); 2. Codon 8(-AA); 3. IVS-2-1(G-A); 4. IVS-1-5(G-S); 5. IVS-1-6(T-S) + , 40 , , 34 22 «0», – «+». . 34 , 17 - -IVS-1110(G- ), -codon8(-AA), -IVS-2-1(G-A) + -IVS-1-5(G-S) , 8– + – -IVS-1-110(G- ), 00 codon8(-AA) -IVS-2-1(G-A) , 9- – . 14 . 16 , , . 0 0 7, 2008 17 -IVS-1-110(G- )/ 0codon 8(- ) - +-IVS-1-110(G- )/ +-IVS-1-110(G). 7 . 47 25 , 20 + . - 9 , + 0 -IVS-1-110(G- ) -IVS-21(G- ) , – + -IVS-1-110(G- )/ +-IVS-1-110(G- ), – - +-IVS-1-110(G0 )/ -IVS-2-1(G- ). 12 . . : + -IVS-1-110(G- )/ 0-IVS-2- 1(G- ) – , +-IVS-1-6(T-S)/ 0-codon 8(+ ), +-IVS-1-5(G-S)/ +-IVS-1-6(T-S) + IVS-1-5(G-S)/ -IVS-1-110(G- ) – , +-IVS-10 110(G- )/ -codon 8(- ) – . , , - , , . : 1. . : . .: , 2002. : http://med.pfu.edu.ru/_new/russian/win/librar y/gematologia/nasl.htm 2. . : . . – .: . 1981. 256 . 3. . : . .: . 2006. 480 . 4. . . : / .: . 2005. 364 . 5. . . .: . 1978. 546 . 6. ., . . . « », 2003, , 62. 7. . . , . 118/5, 5.2; 4.05.2006 . 8. ., ., . . : ( ). , , , , ,. - - : . 2002. 26 . 9. Beutler E. G-6-PD: Population genetics and clinical manifestations // Blood Reviews. 1996. 10: 45-52. 10. Cao A. A world-wide evaluation of one on going research and future directions toward preventing thalassemia and sickle sell disease / 2nd International Thalassemia Summer School. 01-05 April 2002, Kurenia/North Cyprus. . 7-8. 11. El-Beshlawy A., Kaddah N., Moustafa A. et al. Screening for thalassaemia carriers in Egypt: significance of the osmotic fragility test // Eastern Mediterranean Health Journal. 2007. Vol. 13. No. 4. . 780-786. 7, 2008 18 12. Leung T.N., Lau T.K., Chung T.Kh. Thalassaemia screening in pregnancy // Current opinion in obstetrics & gynecology. 2005. .129-134. 13. Medina M. D., Vaca G., LopezGuido B. et al. Molecular Genetics of Glucose-6-Phosphate Dehydrogenase Deficiency in Mexico // Blood Cells, Molecules, and Diseases. 1997. 23(5) Mar 15. . 88-94. 14. Modell B. The ethics of prenatal diagnosis and genetic counseling // World Health Forum. 1990. 11. . 179. 15. Morengo-Rowe A.J. Rapid electroforesis on cellulose acetate. // J. Clin. Pathology. 1965. 18. P. 790. 16. Rasulov E. Express-methods of hemoglobinopathy dignosis in newborns // Journal of molecular genetics, microbiology, virusology. 1990. 1. P. 27. 17. Saiki R. K., Scharf S., Faloona F. et al. Enzymatic amplification of beta-globin genomic sequences and restriction site analysis for diagnosis of sickle cell anemia. // Science. 1985. 230. . 1350. THE ANALYSIS OF HEREDITARY INFRINGEMENTS OF SYSTEM OF BLOOD IN THREE REGIONS OF AZERBAIJAN Akperova G.A. The Baku State University, Baku, Azerbaijan The analysis of hereditary infringements of blood among population Mugan, Shirvan and Western regions of Azerbaijan is carried out. The 65 defective of -globins genes, five types of mutations and three genotypes on -thalassaemia is identified. Two phenotypes on deficiency of enzyme glucose-6-phosphate dehydrogenase at newborns with haemolytic anaemia are established. Cases of disease by hemophilia are fixed. 7, 2008