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, -

, - (. 31.21). , -.

. 31.21. , , -. - - -.

. -, S- .

, - -. - - - . 23 .

L- S- ( ) (. 31.22). S- S-. S-- L- . , (. 31.23). L- , . (. . 29). - (. 31.24). - -, - (, -) --.

. . . 31.2.

. 31.22. , .

(. )

. 31.24. - -, .

(. )

. 31.23. -.

,

, L-, L- L--, . (. 31.25 31.26). (), () (). . , . 31.26, . , . 31.26-31.29.

. . ( 1) L-- , , . L-a- -, .

. --. ( 2) - - -- (. . 18). a- , - ( ), --- ( ) - ( ).

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

. 31.25. . 13 ; . , : 2- ( ), 3 - ( ).

a- -- (-).

. , - -. ( 3) - . , - . ; , ; a- . .

, (. 31.27)

--. , ,

. 31.26. , . , 2 3 . (. ).

, (.. ) 1 . ( 4L, . 31.27) -. --.

( 6L, . 31.27), , (. . 28).

, , , . , 1 1 1/2 - (. . 28).

, (. 31.28)

4V: -. , , , 49 .

5V: - ( 6V, . 31.28), - ( 5V, . 31.28). , ; - --.

6V: -. ,

. 31.27. --, L- (. . 31.26). , -O2 . 20.4.

NAD+- - ( 6V, . 31.28) . .

7V: . : - ( 7V, . 31.28), , . 31.28 ( 8V-10V), - -. --, , , . - - ( 8V-10V, . 31.28). ( 10V, . 31.28) - , --. , -- (. 31.29). ( ) ; , , , . 2 - . - . -- , , , .

, (. 31.29)

, , ; . . -

, - - (. 31.29).

41: -. ( 4V, . 31.28). .

. 31.28. -, L- (. . 31.26). - - -, - - -.

51: ----. 5V (. 31.28). - .

61: --. 2 3 -- - - -. , - () - (), - .

(, )

. ( ). 50 . 510 . ,

. 31.29. -, L- (. . 31.26).

( ) .

A. . ( ), - ( 1, . 31.26). ( 1, . 31.26) .

. . , . , , a-. . . -, -.

. , . . . . .

a-, - -- ( 2, . 31.26). . .

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

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

, , , . , , , .

. . , , ; . . -- ( 3, . 31.26). , .

, (, 12)

- (. 31.21) (. 31.26 31.29), (. 31.23), . ( ) (. 31.21 31.28, , , .

, -

- -, , 12 . .

. . -- . .

. . . , . () (1 ). , , ( 25 -1), [14]-. 5- 10% . 10000 5- . . , , -, 5- .

. (Wellner and Meister, 1981).

Bremer H.J. et al. Amino Acid Metabolism: Clinical Chemistry and Diagnosis, Urban and Schwarzenberg. 1981.

Cooper A.J.L. Biochemistry of the sulfur-containing amino acids, Annu. Rev. Biochem., 1983, 52, 187.

Felig P. Amino acid metabolism in man, Annu. Rev. Biochem., 1975, 44, 933.

Frimter G. W. Aminoacidurias due to disorders of metabolism.

(2 parts), N. Engl. J. Med., 1973, 289, 835, 895.

Paxton R Harris R. A. Isolation of rabbit liver branched chain a-ketoacid dehydrogenase and regulation by phosphorylation, J. Biol. Chem., 1982, 257, 14433.

Paxton R., Harris R.A. Regulation of branched-chain a-ketoacid dehydrogenase kinase, Arch. Biochem Biophys, 1984, 231, 48.

Rosenberg L. E., Scriver C. R. Disorders of amino acid metabolism, Chapter 11. In: Metabolic Control and Disease, Bon-dy P. K., Rosenberg L. E. (eds), Saunders, 1980.

Schwarz V. A Clinical Companion to Biochemical Studies, Freeman, 1978.

Stanbury J. B. et al. (eds), The Metabolic Basis of Inherited Disease, 5th ed., McGraw-Hill, 1983.

Wellner D., Meister A. A survey of inborn errors of amino acid metabolism and transport in man, Annu. Rev. Biochem., 1981, 50, 911.

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