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

?

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

??????:

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

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

????:

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

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

??????:

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

????:

???? ?????? ?????, ?????? ????? ??? ???? ????? (????? ????? ???? ???? ??? ?-50 ????????/????, ???? ????? ?-4-6 ???????/???? ?? ??????). ??? ??? ??? ?? ???? ???? ???? ??????? ????????? ????? ????? ???.

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! ???? ????? Man-6-P ????? GDP in vivo, ????? ????? ???? ?? ?????? ???? ??????????????? ?????????????.

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

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

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

??????:

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

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

????:

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

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

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

??????:

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

????:

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

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

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

??????:

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

????:

????? ??????? ?????? ????? ???? (????? ??? ??????? ????) ?????? ??????? ??????????? ?????? ?????? ???????? (???? ??? ?-CDG Ib MPI).