2: , , , () , .
3: ( ), ,
: ( )
4: , .
, . , , . . . 1 , , , .
5:
.
1) 60- ( ) : . () (), , (MIS, (Mullerian-inhibiting substance)), . : .
1959 , - . - , , Y- - . : Y- , .
6: : , Y-, , , , . (GSD) , (ESD) (CSD).
7: . ( ) ( ) . . . MIS(Mullerian-inhibiting substance), .
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. 1. :
;
. (). . 6 , 12,5- . , , , , .
8: , , XY-. , Y- , , . Y-, (TDF) .
. 2. : ; . 1 . ; 2 . ; 3 ( ). TDF. ( ), ( ); 4 . MIS (Mullerian-inhibiting substance) . ( ), . .
9:
- , Y- , - TDF(Testis determining factor). . 3 Y- Homo sapiens.
. 3. (TDF) Y- :
1959 Y- (23% );
1966 TDF ;
1986 1 ;
1987 11 . ZFY(Zinc Finger Protein, Y-Linked);
1989 , ZFY;
1990 TDF SRY(sex-determining region Y). , , - HMG- (High-Mobility Group)(80 , 320 ..)
XX XY. 17 Y- TDF. : XX-XXSxr XXSxr'. Y- ( ) (PAR) -. , - . (HY-), TDF. XX, , , 1: 20 000.
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10: 1987 , XX, 280 ... Y- , XY (), 22, , TDF Y- Eutheria ZFY. ZFY ZFX -, , , . , , - -, . : , ZFY-zfy1 . ZFY TDF ( Metatheria, ), , Eutheria ( ), Y- . , - zfy1 . ZFY- , .
11: SRY (Sex determining Region Y gene). +:
SRY (HMG-), 80 ;
;
HMG- XY ;
14 ... , , ( ) XX-. , ;
, HMG- SRY-, , . , ;
, SRY- MIS- , .
HMG- SRY. , . SRY .
SRY ( 20 ), SOX (SRY type HMG box). . Sox1, Sox2 - Sox3 . Sox4 -, Sox5 . Sox9 , CD- (campomelic displasia).
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12: ,
, , . , . : Gdy (growth and development), Sxr- Y- .
WT1 (Wilms tumor) , : , , 9- , .
13: Sox9 (SRY type HMG box), 11 17 , CD- , . 13- , SRY MIS (Mullerian-inhibiting substance), .
100 XX , , SRY, Z. : , SRY. Zi, ().
14: DAX1, Xp21. . , DAX1 SRY . - .
SF1, . SF1- . (MIS) .
15: Tda1b (testis-determining autosomal), TDF(Testis determining factor), XY. , SRY, . , (MIS, P450-, 17(α)-), . - , , Sry, Tda1b.
Ta s (Testis associated sex reversal gene), 17, XY- .
:
16: Xq11-12 AR, ( Tfm), .
XY- , : ( )- .
17: - XX- .
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Polled, XX-.
18: , TDF-SRY .
. 2.
19: Y-
- , :
1) ;
2) , ( );
3) ;
4) - (PAR).
20: Y-, , 23% . .
, X- . , : ZFX / ZFY, SOX3 / SRY, UBE1X / UBE1Y, SMCX/SMCY.
Y- .. Y- - . Sox3 UBE1 , ZNF . Y- Sox3 /SRY , UBE1/UBE1Y ZNF/ZNY . X- Y- , .. UBE1Y , , (SMCY, YRRM ..) .
- , , .
Y-, , , . . , , Y- . .
21:
, Gdy (growth and development), Y- .
() : 1) ;
WT1 (Wilmstumor 1) SF1 (steroidogenic factor 1). WT1, 11- , , - . SF1 , , . - . . .
2) ; 3) .
SRY (sex determining region of the Y) DAX1 (DSS-AHC critical region of the X). , SRY, Sox-9 (SRY-box related 9), SRY. . , , SRY. , Tda (testis determining autosomal gene) Tas (Testis associated sex reversal gene) , .
. - DSS (dosage sensitive sex reversal). DAX1 ( ) SRY XY. , DAX1 . . , SRY, , . Wnt4a. , , , .
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22:
SRY- Y-. (Myopus schisticolor .) - -, Y- . Y-. , , , . , , . XY- , . ZW/ZZ- XX/XY- . , . , . . .
:
.
( , , )
. , , . LH LHRH , - LH.
.