?????? ?? ????????, ??? ???? ??????? (C ? H ?? O ?) ???????, ???? ??????? ?? ??? C-2 ???????? ?? (???? ???? ????? ???????? ??????????????? ??????? ????? ????? ??), ????? ????? ?????????? ????? ?? ????? ??????????? ????????????? ?????? ???? ??? ????? ??. ??? ?????????? ??? ???????????????? ????? ?????????? ???????? ?????? ???????????? ?????????? ?????:

?

1. ?????? ???????

????????:

????????? ???????: ?? ??????? ?????? ???? ????????? ?????????? ??? ??? SGLT1 (??????? ???????? ?? ?????????? 1) ????? ???????????? ????????? ????. ?????? ??????? ???????? ??? ? (> 95%), ?? ???????? ??????? ????? ?? ??????? ???? ???? ?? ?????.

??????? ??? ?????????? ?? ??? ????.

??????:

?????????? ???????: ??????? ?????? ???? ?????? ???? (???? ??? GLUT5 ?? ? ?? ???? ?????? ???? GLUT ????? ??????????? ??????? ??). ?????? ??????? ??? ???? (10-20% ???), ?? ??????? ???? ?????? ???????? ??? ????????? ????? ?? ??? ?????? ?????????? ????? ?????? ?? ???? ??? ????? ????.

2. ??????? ????? ????.

????????:

?????????? ???, ?? ??????? ????? ???? ??????? ????? ?? ??? ?????? ??????? ???? ????? ??????? ??? ???.

??????:

?????? ???????? ??? ?? ??? ??????? ?????? ?????????? ???????? ???? (???????? ??????? ???????? ?????????? 50 μmol/L ??? ?? ??? ?? ???????? 4-6 mmol/L ? ??? ???????? ????). ?????? ??? ???????? ??????? ???? ?????? ?????? ???? ??????????? ??? ??????.

3. ??? ??????? ?? ?????????? ???????????? ?????? ?????

????????:

??????????? ?? ?????: ???? ???? ?? ??? ??? ????? ???????? ??????? ????? ??????????? ???? ????? ?? ???????? ??? ???? (GLUT4 ????????? ????).

????????????/????????????: ??? ??? ????? ?? ???, ?? ???????????? (HK) (???????? ???) ?? ???????????? (GK) (??????) ????? ????????-6-???????? (G6P) ??? ??????????? ????. ??? ???? ?????????? ???????????? ????? ???????? ??????.

??????:

??????????? ?? ???????? ???: ??? ??????? ??????????? ?? ??? ??????.

?????????? (MK): ?? ??????? ?????? ?????? ??? ?????????? ????? (?????? ???? ??????? ??? ?? ???? ?????????? ?????) ??????-6-???????? (Man-6-P) ??? ??????????? ????. ??? ?????? ?????????? ???????????? ??????? ??????? ???? ??? ???????.

???????????? ?????????? (PMI): ???????????? ?????????? ????? ????-6-P ????????-6-???????? (F6P) ??? ????????? ??. F6P ??? ????????????? ??????? ??????? ???????????.

4. ??????? ?????????? ?????

????????:

????????????? ????? ??????: G6P ?????? (ATP) ??????? ???? ????????????? ??????? ????? ??? ?????.

?????????? ?????????: ?????? ?? ???? ?????? ?????????? ????????? ?? ???? ?????.

???????? ???????? ??????: NADPH ?? ???????-5-???????? ???? ???? (??? ????????? ?? ?????????????? ????????? ??? ????? ???? ?????? ??).

??? ?????????: ??????????? ??? ???, ?? ???? ??? ????????? ??.

??????:

????????????? ??????? ??? ????????? ???: PMI F6P ??? ????????? ?????? ???, ??? ????????????? ??????? ????? ??? ????? (????? ???? ???????? ??? ????????? ?? ????? ?? ??????? ?????? ???? ????????????? ????????? ?? ?????).

??????????????? ???????????: ??? ??????? ?????? ????? N-????????? ???? ??? ????????? ???? ?????? ???? ?????? ??? ???? ???????! ????-6-?? ???????? ??? ???????????? GDP ?????? ??? ????????? ?? ???? ???, ??????????????? ?? ????????????? ?? ?????? ?????????? ?????? ????????? ???????? ??? ???? ????.

???????????????: ????????? N-????????? ??????????????? ????? ???????? (Man ? GlcNAc ? ????) ?????? ??? ????? ???????????? ????? ??????? ??????. ??? ??????????? ??????????????? ????????? ?? ?????? ??????? ?????? ??? ????????? ?????, ?????????, ?????????????? ?? ??????????????? (?????????, ????? ????????????? ?? ??? ?????? ??? ????) ???? ??? ?????? ??.

????????/?????????? ??? ?????????: ???????????? ??? ?? ???, ???? F6P ?????????? ?? ???? ????????????? ????? G6P ???? ????, ?? ???? ???????? ?? ?????????? ??? ????????? ??, ????? ???????? ???? ??.

5. ????? ???????? ?? ??????????? ?????? ??????

????????:

?????? ???? ??? ????? ???? ????? ??? ???: ??????? ?????? ??????? ?????????.

??????????? ??????? ???? ??? ???????? ?????: ??????????? ???? ??? ??????? ???? ????????? ?????? ????? ??? ??????????? ??????? ????.

??????:

??????? ???? ?????? ???? ???????? ???: ??????? ??????? ????, ???????? ????????? ????? ?????????? ????, ?? ??????????? ?? ??? ??????.

??????????? ??????? ???????? ???????: ?????? ????? ???????? ??????? ???? ?????? ???.

6. ????? ??????????????? ????? ????????

????????:

????? ???????????????: ?????? ??????? ??????? ??????? (????????? ????, ???? ???? ?? ??? ????? ???), ??????? ???? ????????????? ?????? ?????.

??????:

????? ???????????????: ??????????????? ??? ????????? ???? ??????? ????????? ???????????, ??????????????? ?? ????????????? ?? ??????? ?? ???????????????? ???? ??? (??? ??????????, ???? ???????????, ????????????, ????????? ????? ????).

????????? ??????: ??????? ????? ????????? (?????????? ????? ????? ????????).

7. ?????? ?????? ????????

????????:

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

??????:

?????????? ??????? ????? ????????? (??????? ?????? Escherichia coli ????? ?? ???????) ?? ??????? ?????? ????? ??????????????? ???? (CDG Ib MPI ?????? ????) ???? ????????? ?????.