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- , . :

1) ;

2) . , .

, , , ( , . .). .

- - - a- 141 , -146; - (- () . - .

- , . , , , - , . - , , . , , (- ) - ; -.

, , . : ( ) - ( ) - ( ( ) - . ( ), . ( . , , ), .

( ); , (. 6.8). , , 02.

. 6.8. . 100 . ., 40 . ., 20 . .; , , . . , . ( , , .) ( Stanbury J. ., Wyngaardcn J.B., Fredrickson D. S. (editors): The Metabolic Basis of Inherited Diseases. 4th ed. McGraw-Hill, 1978, .)

, 02 . , , 02 02 PQ, . , , , . ., , . . (. 6.8).

. , . (HBF). . . ., . . F , . HBF ; 02, .

, , (. 6.9). , . , . - -, (. 6.10 6.11)

- ( . taut ); R- (relaxed ) (. 6.12). - ; -.

, , . , 0,06 , (. 6.13). .

. 6.9. . , , . ( Stryer L.: Biochemistry, 2nd ed.. Freeman, 1981, .)

, . ; , . (. 6.14). - , .

. 6.10. - - 15 , . . . - - ( )

. 6.11. , - . , . . ( Perutz . F.: Molecular pathology of human hemoglobin. Stereochemical interpretation of abnormal oxygen affinities. Nature 1971:232:408, .)

. 6.12. - R- . ( ), -. , , , ( ). - R- , . , , . , , . - R- , . ( Perutz . F.: Hemoglobin structure and respiratory transport. Sci. Am. [Dec.] 1978:239:92, .)

. 6.13. , . F8. ( Stryer L: Biochemistry, 2nd ed.. Freeman, 1981, .)

. 6.14. , , - .

, . (. 6.15). : - , -, . , . , . . - , . .

. 6.15. . , , , , - . . . -, , . ( ) .

, , , -; (146) (-. , ; (. 6.15).

, - , -. , ( ) (-. R- . , , . ().

2,3-

2,3- (-, ) (. 6.16). 1,3-. . , , . , - -

. 6.16. 2,3-.

-. , -: . , - - , - , - R-.

, , - , . - - .

R- - . ( 3000 /) . , , .

. 6.17. . -. ( Amone A.: X-ray diffraction study of bonding of 2,3-diphosphoglycerate to human deoxyhemoglobin. Nature 1972:237:146, .)

, - -, . ( ), , , . , , .

- - . -, . , - . - - -. , . - R- - , , .

, - ( ), , . . , ( ).

S (Glu Val) , - . , S ( ). , -, (. 6.18). S , . S .

. 6.18. , S ( ) ( ) S. S . , . ( Stryer L.: Biochemistry, 2nd ed., Freeman, 1981, .)

S , , . , S S, S . , - S. ( ), - R- . : S - (- S) , R-.

, S (. 6.18), S , . , R- - S .

S . (. 6.19).

. 6.19. S. ( Maugh . II: A new understanding of sickle cell emerges. Science 1981:211:265, .)

. 6.20. () () . -, , ( ), - (. 36).

: (. 6.20), .

, . - (-) P- (-). , . , (. . 36).

Dean J.. Schechter . N. Sickle-cell anemia: Molecular and cellular basis of therapeutic approaches. (3 parts). N. Engl. J. Med.. 1978. 299. 752, 804, 863.

Kloti I. .. Haney D.N., King L.C. Rational approaches to chemotherapy: Antisickling agents. Science, 1981, 213, 724.

Perutz M. F. Hemoglogin structure and respiratory transport, Sci. Am. (Dec.). 1978, 239, 92.

Perutz M. F. The regulation of oxygen-affinity of hemoglobin: Influence of structure of globin on heme iron, Annu. Rev. Biochem.. 1979, 48. 327.

Stamatoyarmopoulos G. The molecular basis of hemoglobin disease, Annu. Rev. Genet., 1972, 6, 47.

Winslow R.M., Anderson W.F. The hemoglobinopathies. Page 1666. In: The Metabolic Basis of Inherited Disease, 5th ed., Stanbury J. B. et al. (eds.), McGraw-Hill, 1983.

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