1307000400ｨｪｨ 0102, 2008, 030703 44, 6ｧ8 10, 06. 1308ｨC1314 ｨｬ0501 575.17:599.9 ｨｦ03ｨ ｨｦｨｺ040100 ｨ ｨｪ00ｨｦｨｺ00ｨｪｨ ｨｮ000501ｨ 00 05ｨｪ02ｨｪｨｲｨ 04020500000505ｨｪ050004040100 03ｨｦ0000ｨ 04ｨ ｨｬ ｨｹ01ｨｬｨｪｨｦ05 08 2008 6 7. 05. ｨｨ. ｨｨ04090ｸ8apple006 5 1, 04. ｨｺ. 026ｦ001060903076 56ｦ0 2 1 046ｦ0040ｹ00007 0906060200 076 56ｦ00300020006010906 0900060309030403 0300 090ｷ3090006010706 6 700000003090109 ｨｪ07030601076 707 006ｦ0040ｹ000076 707 0ｷ3000003apple6ｦ0 0509 09apple0601076 707 0703 0002000009fl ｨｺ070606090606010706 6ｦ0016ｦ0 00030909 0300 090ｷ3090006010903 006ｦ00401, ｨｪ07030601 634050; e-mail: valery.puzyrev@medgenetics.ru 2 ｨｹ01040306010906 006ｦ0040ｹ0000ｸ806 0ｷ3000003apple 0509 09apple0601076 707 0703 0002000009fl ｨｺ070606090606010706 6ｦ0016ｦ0 00030909 0300 090ｷ3090006010903 006ｦ00401, ｨｹ0104030601 677019 ｨｨ070603040809026ｦ0 6 5 apple00 6ｦ0010ｷ3090ｸ9 01.04.2008 6 7. 046ｦ0 070600076 56ｦ0000909 6ｦ0006ｦ00209096ｦ0 0903076 7076 5 6 700000003090107-0ｹ10809 0003090702076 7090ｹ00006010903 0906060200 076 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5, 016ｦ001 096ｦ00300030902 05. 026ｦ00101000ｸ9060901, 0807fl6 5090206fl 066 50706 0604 0603apple6ｦ003 02fl 0906060200 076 56ｦ00009fl ｨC 6 700000703, 0807 07 0007 03070304, 016ｦ001 0700 00060300 04 016ｦ0apple 090702076 7076 5 ｨC 0600apple 0ｷ300 09 06070604 0ｸ8, 6ｦ0 04 00006 5apple07086ｦ0030702076 7076 5 ｨC 0000apple6 5006ｦ0fl 0609060300036ｦ0 [2]. ｨｪ6ｦ001, 03070200-010402flapple006ｦ0fl 6 70000000309016ｦ0 06036ｦ0026ｦ0 09000603apple04030000030703, 006ｦ0 00076 50703 04apple076 50000 0906060200 040ｸ90ｸ76ｦ0fl 096ｦ0010700070300apple0007060309 0807080402fl0ｷ3090700-000ｸ803 08apple070ｷ3000606076 5 6 5 006ｦ0apple07 07006ｦ006000200000909 09 070809060ｸ86 56ｦ00ｸ90ｸ76ｦ0fl 03070200010402flapple00040ｸ9 0603apple04010304apple04 6 70000076 5, 060706036ｦ06 502fl0ｸ90ｸ70903 6 700-0007020700 0ｸ8 0ｹ00002076 5000ｹ00006010903 0807080402fl0ｷ30906 09 6 700000003090ｹ0000601040ｸ9 070600076 504 07020009000006 0ｹ00002076 500016ｦ0. ｨｦ03000ｹ00006036 50000000ｸ800 0906060200 076 56ｦ00009fl 6 5 07 026ｦ0060309 6 700000003090ｹ00006010706 0ｹ10809 0003090702076 70909 006ｦ0060200 06036 50000000ｸ803 07020009000006 (0403), 0703fl6 7070ｸ700000007060309 (6 7apple04096ｦ0) 006ｦ0060200 06036 50000000706 086ｦ00307-02076 7090006 apple6ｦ00902090ｹ0000ｸ803 0ｹ10300090ｹ00006010903 0807080402fl0ｷ30906 0603apple6ｦ0000ｸ8 0903000ｸ903 066 5070ｸ9 09060307apple090ｸ9. 05 1968 6 7. 030007apple0003090ｹ00006010700 07 070600076 56ｦ0000900 6ｦ00000076 707 006ｦ0040ｹ000076 707 006ｦ008apple6ｦ06 502000009fl 0ｸ80207 08apple00 06036ｦ06 502000007 04.ｨｨ. 03070ｹ001076 50ｸ803 09 04.05. ｨｪ090307020000-6 50ｸ803-ｨｺ0006076 506010903, 0703030003096 5040903, 0ｹ00307 060703apple6ｦ0000000090ｸ9 0304-036ｦ00ｷ30906, 08apple096 507 fl0ｸ70903 01 086ｦ0030702076 70909 09 006ｦ0010708020000090ｸ9 0903 6 5 0708apple00 00020000000ｸ803 0ｹ1030007066ｦ003, 0608070607 06036 5040003 066 5070007 apple6ｦ0090900 0ｹ1010702076 7090ｹ00006010903 026ｦ0010307apple076 5, 03apple6ｦ0 090ｷ3090700000ｸ803 0401026ｦ0 076 5 0809090009, 070607 00000007 apple0008apple07 040103096 500076 707 08076 500 000009fl, 6ｦ0 0906-060200 076 56ｦ00009fl 6 5 0ｹ1030703 006ｦ008apple6ｦ06 50200000909 03076 70403 060ｸ86 7apple6ｦ00300 6 56ｦ00800040ｸ9 apple070200 6 5 070301apple0ｸ8030909 00076 50ｸ803 6ｦ0020200020006 6 70000076 5 [3]. 05060107apple00 05.ｨｨ. ｨｴ02apple070903060700 (1970), 07 060408 6ｦ0fl 08apple07 0200-0304 6 7000300apple076 700000007060309 0403, 6 50ｸ806016ｦ0096ｦ002 08apple00 080702070800000900 07 030700070304036ｦ000030007060309 070200090009 02fl 016ｦ008 0706 6 700076 7apple6ｦ002090ｹ00006010706 0907000ｸ8 090209 6ｦ00800 0600030009 [4]. ｨｨ00apple6 56ｦ0fl 0703000ｹ000-06036 50000006ｦ0fl 0804 0209016ｦ00ｷ309fl 04.ｨｨ. 03070ｹ001076 56ｦ0 09 06076ｦ06 503. 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[26]. ｨｦ6 7apple6ｦ000090ｹ0090306fl 0ｹ103090309 6 50403fl 08apple090300apple6ｦ00309 0ｹ10300070608000ｷ309-02090ｹ00006010903 006ｦ0060200 06036 50000000ｸ803 096ｦ0 0702006 56ｦ0000906 ｨC 006 5apple0006060109-0309 09 0209000601090309. ｨｪ6ｦ0010903 07 apple6ｦ0090703, 0307080007 04036 500apple08 6ｦ00300, 0ｹ00307 02000007030000 0ｹ1030703 08076 50600030006030000 09 08apple00 0807026ｦ06 76ｦ00003 02fl 0300076 70903 apple046 70903 09090702fl03076 5 [7, 27]. 05 096ｦ001020ｸ90ｹ000000900 060200 04-0003 02090400 096ｦ0030003090300, 0ｹ00307, 0807-6 509 0903070304, 6 5 070300070400000909 030000 0002006 506010903 096ｦ0 0702006 56ｦ0000906 6 5 006ｦ0060307fl0ｸ70000 6 5apple0003fl 0601026ｦ0-0ｸ86 56ｦ0000306fl 036ｦ0016ｦ0fl 0800 060903046ｦ00ｷ309fl, 016ｦ001 09 06 01026ｦ00606090209016ｦ00ｷ309-0006 000003apple070307060703000ｸ803 6 5apple0708 0000000ｸ803 0807apple0701076 5 apple6ｦ0096 5090309fl, 070600076 56ｦ000000706 006ｦ0 02000007030908090ｹ00006010903 08apple07fl6 502000009fl03. 06000007-030908090ｹ00006010700 070809066ｦ0000900 0607030000 060900 apple07036ｦ00200000ｸ803 0807apple07-01076 5 apple6ｦ0096 5090309fl, 0ｹ00307 07016ｦ0096ｦ00207 6 56ｦ008000700 6 50209fl000900 006ｦ0 036ｦ0010309-0104 01020900090ｹ0000601076 707 6 500 000009fl 070200000ｸ803 09 6 700000003090ｹ00006010700 010700060402000309apple076 56ｦ0000900 0600030006 0ｹ1030903 070200000ｸ803, 06036ｦ00207 6ｦ001-03096 50007 0708070200fl030006fl 066 500 000009fl0309 0909 03070200010402flapple000706 090702076 70909, 0207apple0309apple04fl 00076 50ｸ800 6 7apple0408080ｸ8 096ｦ0 0702006 56ｦ0000906. ｨｴ030703 08040300 0609060300036ｦ003090109 006ｦ0060200 06036 50000000ｸ803 07020009000006 0ｸ802 07 0709006ｦ00ｹ00000 6 5 1960-00 6 707 0ｸ8 05. 026ｦ00101000ｸ90609010703 016ｦ001 ｰapple6ｦ009 000200000900 09 07 ｨB00 090000000900 ｱ ( ｰlumping and splitting ｱ) 006ｦ0 070600076 500 6ｦ000000ｸ803 0807 03070200010402flapple000706 6 700000003090100 02000007-030908076 5, 0403070ｹ000fl0ｸ90ｸ70903 0903 08apple09006ｦ0 0200080007060300 01 030706 090209 09000706 ｰ6 700000003090ｹ00006010706 0007090702076 70909 ｱ [28]. 05 0ｹ1030703 06030ｸ8060200 6ｦ000000ｸ800 0807 03070200010402flapple000706 6 700000003090100 030000 0002006 506010903 02000007030908076 5, 0807 01070307apple0ｸ803 06apple6ｦ06 500096 56ｦ0000306fl 0608000103apple 006ｦ0060200-06036 50000000ｸ803 07020009000006 6 5 apple6ｦ00902090ｹ0000ｸ803 0ｹ10300070807080402fl0ｷ309fl03, 06036ｦ000076 5fl0306fl 020903090309apple040ｸ90ｸ7090309 6 5 07016ｦ0096ｦ0030002000007060309 6 50ｸ8-06016ｦ0090ｸ86 56ｦ000030ｸ803 080702070800000906 07 070607 000000070603fl03 0807080402fl0ｷ309-0700000ｸ803 08apple070ｷ3000606076 5 09 096ｦ0010700070300apple000706030006 0207apple0309apple076 56ｦ00009fl
131310 ｨｨ04090ｸ8apple006 5, 026ｦ001060903076 56ｦ0 6 7apple04096ｦ0 006ｦ0060200 06036 50000000ｸ803 07020009000006. 0508apple6ｦ06 500 02096 507 070303000ｹ0000007, 0ｹ00307 03070200010402flapple0007-6 700000003090ｹ00006010900 030006030ｸ8 fl6 502fl0ｸ903-06fl 016ｦ00ｹ00006036 50000000ｸ80309, 03070ｹ0000ｸ80309 09 07 000709006ｦ00ｹ0000ｸ80309 6 5 06apple6ｦ06 5-0000000909 06 01020900090ｹ0000601090309 08apple0909006ｦ0016ｦ00309, 01070307apple0ｸ800 070809060ｸ8-6 56ｦ00ｸ90306fl 010702090ｹ00006036 500000007, 0807apple0706 00 6 56ｦ0 0402076 509030ｸ8, apple6ｦ00902090ｹ0000ｸ8 09 0000060403 07030ｹ0000302096 5040ｸ9 0604 ｨB000103096 500040ｸ9 070ｷ300000104 [29]. 02ｨｦ000001ｨｬ00ｨｹｨｺ0402ｨｹ 07000400ｨｪｨ 0102 ｨｹ01ｨｬｨｪ0501ｨ 07 04020500000505ｨｪ050004040107 03ｨｦ0000ｨ 040009 ｨｹ01040306010700 0107apple0000000700 006ｦ006000200000900 0909040ｹ0000007 0807 07020004070304 0608000103apple04 6 700000003090ｹ00006010903 036ｦ0apple0100apple076 5. 05apple00 09 000903 6 700000003090ｹ00006010900 036ｦ0apple0100apple0ｸ8 060906030003 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000200000ｸ803 02000007030908076 5 0807 036 500apple 090207 6 50ｸ8040009090207080000000ｸ800 0807020708000009fl. 05 036ｦ0 02090ｷ300 08apple00 06036ｦ06 50200000ｸ8 030000 0002006 506010900 020000070309080ｸ8, 01070307apple0ｸ803 006ｦ00309 08apple09066 507000007 006ｦ0096 56ｦ0000900 ｰfl01040306010903 006ｦ0-060200 06036 50000000ｸ803 07020009000006 ｱ. 05080900070ｷ300apple00 000202flapple006ｦ0fl 6ｦ0036ｦ0010609fl, I 030908 (050902 1) ｨC 006ｦ0060200 06036 50000000700 6ｦ00403070607030007-070309006ｦ00003000700 096ｦ0 070200-6 56ｦ0000900, 070607 000000070603fl0309 01020900090109 01070307apple076 707 fl6 502fl000306fl 080709 000000 08apple07fl6 50200000900 (01 20ｨC40 6 707 6ｦ003) 09 070200040706 080702090307apple02090903 06070ｹ000036ｦ00009fl 08apple076 7apple00060609apple040ｸ90ｸ70006 030709-08000ｹ001076 50706 09 0809apple6ｦ00309 000706 060903080307036ｦ003090109. 0507 0600apple00-09000ｸ8 70-03 6 707 076 5 XX 6 5. 6 5 ｨｹ0104030909 070200090000 0703000706090209 01 ｰ03070908000ｹ001076 50706 0207apple0300 ｱ 6 509020ｸ9060601076 707 0ｹ1000ｷ300026ｦ00207-0309000209036ｦ0 [40, 41]. 050ｸ80607016ｦ0fl apple6ｦ00608apple070603apple6ｦ00000000007060300 096ｦ0-0702006 56ｦ00009fl (38.6 006ｦ0 100 030ｸ806. fl010403076 5 0807 06apple6ｦ06 5000000090ｸ9 1ｨC2 : 100 030ｸ806. 6 5 0309apple076 50703 006ｦ006000200000909) 6 5 ｨｹ0104030909 0ｸ8-026ｦ0 070ｷ3000000006ｦ0 016ｦ001 ｰ0609 09apple06010906 070ｹ06ｦ06 7 ｱ 006ｦ001070802000009fl 096ｦ0-0702006 56ｦ00009fl, 01apple0408000006040906 6 5 0309apple00 [17, 18, 35]. 020702000104-02flapple000706 070600076 50706 096ｦ0 0702006 56ｦ00009fl fl6 502fl000306fl 046 50002090ｹ000000900 04 fl010403076 5 0ｹ00906026ｦ0 03apple090004010200070309 000ｸ803 CAG-08076 50307apple076 5 07 39ｨC71 0807 06apple6ｦ06 5000000090ｸ9 06 19ｨC36 6 5 0007apple0300 6 5 6 7000000 SCA1, 0207-016ｦ0020909076 56ｦ000000ｸ803 6 5 07 026ｦ0060309 6p22ｨCp23 [18]. 05apple00 09 apple6ｦ009-02090ｹ0000ｸ803 0207apple03 006ｦ0060200 06036 50000000ｸ803 6ｦ0036ｦ001060906 6 5 ｨｹ0104030909 050902 1 6 50603apple000ｹ06ｦ0000306fl 6 5 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3'-000003apple6ｦ000060209apple0400030706 0ｹ06ｦ0060309 6 700006ｦ0 D02. ｨｮ09060207 CTG-08076 50307apple076 5 6 5 0007apple0300 060706036ｦ06 502fl0003 0703 5 07 37, 08apple09 08apple000304036ｦ00ｷ30909 ｨC 0703 37 07 50, 08apple09 086ｦ0030702076 70909 ｨC 07020000 50 09 07 00000601070200010903 030ｸ806fl0ｹ0 CTG-08076 50307apple076 5. ｨ 06060200-076 56ｦ0000900 080702090307apple020909036ｦ0 CTG-08076 50307apple076 5 6 700006ｦ0 030907-030700090008apple07030009000109006ｦ0090ｸ8 04 070200000ｸ803 ｨｹ0104030909, 08apple09006ｦ0-0200086ｦ00ｸ70903 01 apple6ｦ009000ｸ803 0ｹ10300090ｹ00006010903 6 7apple0408086ｦ003 (fl0104030ｸ8, 0ｹ16 500000109, 0ｹ16 500000ｸ8, 0ｸ9016ｦ06 709apple0ｸ8 09 07026 76ｦ0000ｸ8), 07 006ｦ0apple0408090207 000909010906 04apple076 5000000 6 700000003090ｹ0000601076 707 apple6ｦ009000707 apple6ｦ00909fl 6 5 06apple6ｦ06 50000000909 06 0807080402fl0ｷ309fl0309 006 5apple6ｦ0090909 09 000909010700 0607-00apple086ｦ0000900 000006036ｦ0 090200000ｸ803 6ｦ0020200020006 [33, 34]. ｨｮ6ｦ0060307036ｦ0 0205 6 5 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1304020500000505ｨｪ050004040100 03ｨｦ0000ｨ 04ｨ ｨｬ ｨｹ01ｨｬｨｪｨｦ05 1311 6 70702076 5000706 0702000ｸ9, 040306036 50000000706 070306036ｦ002070603000ｸ9 apple6ｦ00902090ｹ0-000706 06030008000009 6 50ｸ8apple6ｦ00800000007060309, 08076 50ｸ8040000000ｸ803 04apple076 5000003 0300036 70003076 70207 09006ｦ0 09 0000 0706036ｦ003070ｹ000070603000ｸ9 0300036 70003076 70207-0900apple00 0401036ｦ0090ｸ8 6 5 01apple076 509. ｨｺ6ｦ00902090ｹ06ｦ00ｸ903 6 500 0207apple030ｸ8 000209-0ｷ309036ｦ0 0200apple030000036ｦ0 0300036 70003076 70207 0900apple00 0401036ｦ0090ｸ8. ｨｨapple09 0207apple0300 I 04ｨｴ02 0903000ｸ90306fl 030702000107 060903080307030ｸ8 0307apple070407 0800apple0000070609030706 0300036 70003076 70207 090000030909, 09 0200apple03000003000ｸ806 0002000103 066 5fl096ｦ000 06 0ｹ1apple0903apple070ｷ309036ｦ0apple000706 apple6ｦ006036 507apple09030706 0ｷ309030703apple0703-b5-apple00 0401036ｦ0090706 ( 096ｦ00207apple6ｦ0090706). ｨｨapple09 0207apple0300 II ｨC 040306036 50000006ｦ0fl 070306036ｦ00207060300 06 6 7000000apple6ｦ0020909076 56ｦ000000ｸ803 0200apple- 03000003000ｸ803 00020001030703, 096ｦ003apple6ｦ06 7096 56ｦ00ｸ90ｸ70903 016ｦ001 apple6ｦ006036 507apple09-03040ｸ9, 036ｦ001 09 030901apple070607036ｦ0020000040ｸ9 0ｷ309030703apple0703-b5- apple00 0401036ｦ0-0904 6 5 0ｹ1apple0903apple070ｷ309036ｦ003 09 02000601070ｷ309036ｦ003. ｨｨapple09 0002090ｷ3090300 NADH-0ｷ309030703apple0703-b5-apple00 0401036ｦ0090ｸ8 07 apple6ｦ009040ｸ90ｸ7090606fl 030003-6 70003076 70207 0900 0000 0800apple000307 0903 07 apple6ｦ0030007 6 5 6 70003076 70207 0900, 09 04apple076 5000000 0300036 70003076 70207 09006ｦ0 6 5 01apple076 509 04 086ｦ00ｷ309000003076 5 06 6 707-030709096 70703000706 0207apple030706 6 5 06apple00 000003 060706036ｦ06 502fl0003 0701070207 18% (04 6 7000300apple0709096 70703 ｨC 2%, 6ｦ0 6 5 0007apple0300 ｨC 030000000400 1%). 04ｨｴ02 fl6 502fl000306fl apple0009040200036ｦ0030703 0304036ｦ00ｷ30906 6 700006ｦ0 096ｦ00207apple6ｦ0-090ｸ8 (DIA1), 0207016ｦ0020909076 56ｦ00000076 707 6 5 0300apple0309006ｦ00200000706 07 026ｦ0-060309 02090000076 707 0802000ｹ06ｦ0 03apple0703070607030ｸ8 22, 6 5 040ｹ06ｦ006030100 q13-qter. 05 006ｦ0060307fl0ｸ70000 6 5apple0003fl 09096 50006030007 0701070207 30 0304036ｦ00ｷ30906 6 5 6 700-0000 DIA1, 19 0909 01070307apple0ｸ803 08apple096 507 fl03 01 apple6ｦ0096 50903090ｸ9 04ｨｴ02 I 0309086ｦ0 [16]. 05 0309apple00 04ｨｴ02 0ｹ06ｦ0060307 6 50603apple000ｹ06ｦ0000306fl 06apple00 09 0ｹ1060109030706076 5 0202fl060109 [52], 00 0900090ｹ0000ｸ800 0602040ｹ06ｦ009 070809066ｦ0000ｸ8 6 5 apple046 70903 0807-080402fl0ｷ309fl03 0309apple6ｦ0. 05 ｨｹ0104030909 000ｸ700 6 5 1970-00 6 707 0ｸ8 0ｸ802 6 50ｸ8fl6 5020000 01apple0408000006040906 070ｹ06ｦ06 7 04ｨｴ02 [53]. 05 006ｦ0060307fl0ｸ70000 6 5apple00-03fl apple6ｦ00608apple070603apple6ｦ00000000007060300 04ｨｴ02 6 5 ｨｹ0104030909 070ｷ30000096 56ｦ0-000306fl 016ｦ001 1 : 5677 [15]. ｨｨ00apple6 56ｦ0fl 0807080ｸ803016ｦ0 03070200010402flapple0007-6 700000003090ｹ0000601076 707 0906060200 076 56ｦ00009fl 036ｦ0apple6ｦ0010300apple6ｦ0 0304036ｦ00ｷ30909 6 700006ｦ0 DIA1 04 fl01040306010903 070200000ｸ803 04ｨｴ02 I 0309086ｦ0 07016ｦ0096ｦ0026ｦ00600 0009-0406080004000706 [14]. 0409 07 006ｦ0 0909 09096 5000603000ｸ803 0304036ｦ00ｷ30906 6 70005apple6ｦ06 5000903000200000ｸ800 6ｦ000000ｸ800 0807 08fl0309 006ｦ0060200 06036 50000000ｸ803 0702000900fl03 04 fl010403076 5 09 6 5 0309apple00 046ｦ0096 56ｦ0000900 070200090009 (OMIM*) ｨｪ0908 006ｦ0060200-076 56ｦ00009fl** 070000 0204036ｦ00ｷ309fl 04 fl010403076 5 ｨｺ6ｦ00608apple070603apple6ｦ00000000007060300 070200090009 006ｦ0 100 030ｸ806. 0ｹ00002. 00090300apple6ｦ00304apple006ｦ0fl 06060ｸ802016ｦ0 04 fl010403076 5 6 5 0309apple00 05080900070ｷ300apple00 000202flapple006ｦ0fl 6ｦ0036ｦ001-0609fl I 0309086ｦ0 (164400) 020907030700090ｹ00006016ｦ0fl 090603apple070209fl (160900) ｨｦ01040207026ｦ0apple09006 7006ｦ00200006ｦ0fl 030ｸ8-04000ｹ0006ｦ0fl 090603apple070209fl (164300) 046ｦ0060200 06036 50000006ｦ0fl 0ｹ100090903070800-00090ｹ00006016ｦ0fl 0300036 70003076 70207 090000-0309fl (250800) 0205 ATXN1 ｨｴ0106086ｦ0000609fl CAG-08076 50307apple076 5 36.8 1.0ｨC2.0 [42, 35] 0205 DMPK ｨｴ0106086ｦ0000609fl CTG-08076 50307apple076 5 21.3 4.0ｨC5.0 [44, 43] 0205 PABPN1 ｨｴ0106086ｦ0000609fl GCG-08076 50307apple076 5 8.8 1.0 [36, 45] 02ｨｺ DIA1 Pro269Leu 14.9 1.0 [16, 15] 3-02 060900 apple0703 (273750) 02ｨｺ CUL7 4582insT 10.0 040009096 50006030007 [37] * OMIM ｨC 00070300apple 6 5 0ｹ102000103apple0700000706 6 500apple060909 016ｦ0036ｦ002076 76ｦ0 05. 026ｦ00101000ｸ90609016ｦ0 ｰMendelian Inheritance in Man ｱ (MIM): www.ncbi.nlm.nih.gov/omim/. ** 0205 09 02ｨｺ ｨC 6ｦ00403070607030007-070309006ｦ00003000ｸ806 09 6ｦ00403070607030007-apple000ｷ3000606096 5000ｸ806 0309080ｸ8 006ｦ0060200 076 56ｦ00009fl. 05 ｨｹ0104030909 096ｦ0 0702006 56ｦ0000900 0ｸ80207 09096 50006030007 0807 006ｦ0-096 56ｦ000090003 ｰ 00030ｸ96 70000 ｱ ｨC 016ｦ001 066 5070007 apple6ｦ009006ｦ0fl 006ｦ0060200-06036 50000006ｦ0fl 0207apple036ｦ0 040200 6ｦ0apple00076 707 086ｦ0apple6ｦ002090ｹ06ｦ0 [47]. 05 6ｦ00200000006040003, 040800 016ｦ001 ｨｦ060205, 0ｸ80209 070809066ｦ0000ｸ8 0007-6 50ｸ800 0602040ｹ06ｦ009 0ｹ1030706 070200090009 [48]. 05 0ｷ300020703 07016ｦ0096ｦ0006ｦ0 6 50ｸ80607016ｦ0fl 0ｹ06ｦ0060307036ｦ0 0701040207026ｦ0apple09006 7006ｦ00200000706 030907 0906-03apple07020909 08apple000903040ｸ70006036 500000007 04 fl0104030601076 707 006ｦ0060002000009fl 06 006ｦ0010708020000090003 0000 6 5 ｨｬ060300-0202 6ｦ00006010703 0402040600. ｨｮ6ｦ0060307-036ｦ0 ｨｦ060205 06apple00 09 fl0104030601076 707 006ｦ0060002000009fl ｨC 1 : 11680, 0ｹ00307 6 50ｸ80400 6 5 10ｨC20 apple6ｦ009 070300070609030002000007 apple006 7090603apple09apple0400-030706 6 5 006 5apple070800 [36]. 070000 ｨｦ060205 0909070209apple076 56ｦ000 6 5 07 026ｦ0060309 14q11.2ｨC13 09 07036 5000306036 50000 096ｦ0 060900030009 08070209-(02)- 066 5fl090ｸ86 56ｦ00ｸ90ｸ7006 707 0002016ｦ0 2 (PABPN1), 060204086ｦ00ｸ7006 707 026ｦ0010307apple0703 080702096ｦ0 00-00090209apple076 56ｦ00009fl 03ｨｺ0401; 09 0000030902090ｷ309apple076 56ｦ0006ｦ0 0304036ｦ00ｷ309fl ｨC 046 50002090ｹ000000900 0ｹ00906026ｦ0 0107080906 03apple090004010200070309 000ｸ803 GCG- 08076 50307apple076 5 6 5 1-03 0ｹ10109070000 6 700006ｦ0 [49]. 05 0007apple0300 6 70000 0607-00apple080903 0400060300 036ｦ000 0003000ｸ803 0107080906 08076 50307apple076 5 GCG, 6ｦ0 04 070200000ｸ803 0ｹ009060207 08076 50307apple076 5 070603096 76ｦ00003 8ｨC13 0107080906. 05 000001070307apple0ｸ803 0807080402fl0ｷ309fl03 0ｹ10106086ｦ0000609fl 0ｹ00906026ｦ0 03apple09-0004010200070309 000ｸ803 08076 50307apple076 5 08apple0709060307 0903 096ｦ0 060ｹ00003 08apple07-0603076 707 07 6ｦ06 502000009fl GCG-08076 50307apple076 5 [46, 50], 6 5 apple046 70903 ｨC 6 50300060300 06 0ｹ10106086ｦ00006090006 GCG-08076 50307apple076 5 08apple0709060307 0903 GCA-6 506036ｦ06 5016ｦ0 [51]. 05 ｨｹ0104030909 6 50800apple6 50ｸ800 08apple076 500 000007 03070200010402flapple0007-6 7000000-03090ｹ00006010700 0906060200 076 56ｦ0000900 40 070200000ｸ803 09 6 50ｸ8fl6 50200006ｦ0 0304-036ｦ00ｷ309fl 6 5 1-03 0ｹ10109070000 6 700006ｦ0 PABPN1, 096ｦ001020ｸ90ｹ06ｦ00ｸ90ｸ76ｦ0fl06fl 6 5 046 50002090ｹ000000909 GCG-08076 50307apple076 5 07 10 [36]. 02 6 5 17 06000300fl03 08apple09 08applefl030703 0600016 5000009apple076 56ｦ0000909 6 700006ｦ0 0406036ｦ000076 5020000 030908 0304036ｦ00ｷ30909 ｨC 6 506036ｦ06 5016ｦ0 0ｹ000030ｸ8apple0003 GCG-08076 50307apple076 5. 046ｦ0060200 06036 50000006ｦ0fl 0ｹ10009090307080000090ｹ00006016ｦ0fl 0300036 7000307-6 70207 0900000309fl I 0309086ｦ0 (04ｨｴ02) ｨC 006ｦ0060200 06036 50000000700 6ｦ0040307-0607030007-apple000ｷ3000606096 5000700 096ｦ0 0702006 56ｦ0000900, 08apple07fl6 502fl0ｸ90ｸ7000006fl 0ｷ3096ｦ00007090703 010708000ｸ803 080701apple076 5076 5 09 060209090906030ｸ803, 07 0ｸ804010706,
131312 ｨｨ04090ｸ8apple006 5, 026ｦ001060903076 56ｦ0 006ｦ0 DIA1 (Len72Pro, Vall105Met, Arg57Gln), 07 006ｦ0apple04-080000000ｸ803 04 0209086ｦ006040903 06070600 0006 fl010403076 5 ｨC fl0807000ｷ3006 5 09 0109036ｦ0060ｷ3006 5, 0000 0ｸ8026ｦ0 09 0000030902090ｷ309apple076 56ｦ0006ｦ0. ｨｨ0709 000000 0909 0ｹ1030706 0800 6 50ｸ8 07apple0109 04 16 0000apple07 06036 50000000ｸ803 fl01040306010903 070200000ｸ803 09 0903 8 09 07apple076 50ｸ803 apple07 06036 50000000901076 5 08apple076 500 0000 6ｦ0006ｦ0020909 0000apple6ｦ06 500076 5000609fl 0807 060ｷ30008020000090ｸ9 096ｦ0 0702006 56ｦ00009fl 06 0ｹ000030ｸ8apple0003fl 036ｦ0apple0100apple6ｦ00309, 02026ｦ0000109apple040ｸ90ｸ7090309 6 70000 DIA1, 6ｦ0 096ｦ0030003 0600016 5000009apple076 56ｦ0000900 6 5060003 006 5fl0309 0ｹ101090700076 5 6 700006ｦ0 [16]. 050ｸ8fl6 50200006ｦ0 00076 56ｦ0fl 03090606000006-0304036ｦ00ｷ309fl 6 5 0ｹ10109070000 9, 08apple096 507 fl0ｸ76ｦ0fl 01 096ｦ003000000 Pro269Leu 6 5 0200apple0300000300 NADH-0ｷ309030703apple0703- b5-apple00 0401036ｦ00900. ｰｨｹ01040306010906 060900 apple0703 0009090107apple07060207060309 ｱ (ｨｹ0504) 090209 060900 apple0703 3-02. 05 036ｦ0010903 006ｦ0096 56ｦ000090003 070809066ｦ000000ｸ806 6 5 2007 6 7. 04.ｨｺ. 026ｦ001060903076 50706 06 06076ｦ06 503. [37] 060900 apple0703 0ｸ802 6 501020ｸ90ｹ00000 6 5 016ｦ0036ｦ002076 7 6 70000076 5 OMIM 016ｦ001 6ｦ002000300apple006ｦ003096 5-000ｸ806 09096 500060300070304 ｰ060900 apple070304 3-02 ｱ (OMIM 273750). ｨｦ0600076 56ｦ0000900 02fl 07 ｨB00 0900000009fl 0ｹ1030903 6 50403 060900 apple0703076 5 0807 07 000903 00070300apple0703 016ｦ0036ｦ002076 76ｦ0 ｨC 07 0900 09 030703 0800 6 70000 CUL7 (010402020900 7), 0007 06 apple6ｦ00902090ｹ0000ｸ80309 0304036ｦ00ｷ309fl0309. 05 37 fl01040306010903 06000300fl03 04 070200000ｸ803 06 08apple070807apple0ｷ30907-006ｦ002000007 000909010903 apple0706030703, 08apple00-09 08070603006ｦ0036ｦ00200000706 096ｦ0-00apple08010706 apple0706036ｦ0, 02090ｷ3006 50ｸ80309 09090307apple0209fl0309 09 0007apple- 036ｦ00200000ｸ803 04apple076 5000003 0607036ｦ0030703apple070800076 707 6 707apple0307006ｦ0 09 0900-030002020001030703 0ｸ802 08apple076 500 0000 0601apple090009006 7 08070200076 707 6 76ｦ008020709 00076 707 6 7000007036ｦ0 [37], 0807016ｦ0096ｦ06 5040906 6 700000003090ｹ000-06010700 060ｷ300080200000900 06 02070104060703 D6S282 (0007-6ｦ00202 24.6). 016ｦ000 09 6ｦ003000ｸ806 apple006 7090700 6 700006ｦ0 0ｸ802 0708apple00 00020000 030008 04 036ｦ0apple0100apple6ｦ00309 D6S1552 09 D6S271 6 5 6 70000 010402020900 7 (Cullin7, CUL7), 07036 5000306036 50000000703 096ｦ0 apple6ｦ0096 509030900 3-02 060900 apple07036ｦ0 09 016ｦ0apple0309apple076 56ｦ000000703 06apple6ｦ06 50009030002000007 0000 6ｦ06 50007 6 7apple0408080706 040ｹ000000ｸ803 0909 00000601070200010903 0603apple6ｦ000 [54]. ｨｨ07-06010702000104 01020900090ｹ00006010900 6ｦ000000ｸ800 fl01040306010903 070200000ｸ803 0ｸ80209 0603070809 06 0102090009010706 apple00 01076 707 060900 apple07036ｦ0 3-02, 0307 6ｦ06 50307apple0ｸ8 apple0004090209, 0ｹ00307 CUL7 fl6 502fl000306fl 0307apple07040903 016ｦ000-09 6ｦ003000ｸ803 6 700000703 02fl fl0104030601076 707 6 56ｦ0apple096ｦ000036ｦ0 006ｦ0000909036ｦ0 09 08apple076 5000209 08applefl030700 0600016 5000009apple076 56ｦ0000900 6 5060003 25 0107 09apple04-0ｸ90ｸ70903 0ｹ101090700076 5 6 700006ｦ0 CUL7. ｨｬ 6 5060003 43 070200000ｸ803 0ｸ8-026ｦ0 09 0000030902090ｷ309apple076 56ｦ0006ｦ0 00076 56ｦ0fl 0304036ｦ00ｷ309fl 4582insT 6 5 6 7000000 CUL7 6 5 6 707030709096 70703000703 0607060307fl000909, 0807096 50702096 5-046ｦ0fl 6 50ｸ806036ｦ06 5090300 03070ｹ0000ｸ806 096ｦ06 7000709 070200000ｸ803, 6ｦ0 04 0903 34 apple07 06036 50000000901076 5 ｨC 0708apple00 0002090300 6 7000300apple0709096 70703000700 0007-06090300020006036 507, 08fl0300 0909 000903 0ｸ80209 09 07apple076 50ｸ8. 3-02 060900 apple0703 (OMIM 273750) 0703000706090306fl 01 6 7apple04080800 006ｦ0060200 06036 50000000ｸ803 060900 apple0703076 5 06 086ｦ0030702076 7090ｹ00006010706 000909-0107apple070602070603000ｸ9. ｨ 6ｦ0 0702006 56ｦ0000900 006ｦ0096 56ｦ00007 0807 0800apple6 50ｸ803 0401-6 56ｦ003 026ｦ00309020906 03apple0003 6ｦ06 50307apple076 5 (Miller, McKusick, Malvaux), 6 50800apple6 50ｸ800 070809066ｦ06 5040903 0ｹ1030703 060900 apple0703 6 5 1975 6 7. [55]. ｨｴ0307 apple00 010700 006ｦ0060200 06036 50000000700 096ｦ0 0702006 56ｦ0000900 06 6ｦ00403070607030007-apple000ｷ3000606096 5000ｸ803 0309080703 006ｦ0060200 076 56ｦ00009fl 036ｦ0- apple6ｦ0010300apple090904000306fl 08apple00-09 08070603006ｦ0036ｦ00200000706 096ｦ0 00apple08010706 apple0706036ｦ0, 02090ｷ3006 50ｸ80309 09090307apple0209fl0309, 0007apple036ｦ00200000ｸ803 0900-030002020001030703 09 apple0000036 700000702076 7090ｹ0000601090309 0909030000000009fl0309 6 5 01070603fl03 (030700010900 03apple04 0ｹ06ｦ0030ｸ800 0107060309 09 020900000ｸ800 0807-096 507000109). ｨｮ6ｦ0060307036ｦ0 3-02 060900 apple07036ｦ0 6 5 0309apple00 000009096 5000603006ｦ0, 036ｦ001 016ｦ001 096ｦ0 30 020003 070809066ｦ00007 6 506006 707 0701070207 50 00 0900090ｹ0-000ｸ803 01020900090ｹ00006010903 0602040ｹ06ｦ0006 5 096ｦ0 0702006 56ｦ00009fl 6 5 apple6ｦ009000ｸ803 0ｹ10300090ｹ00006010903 6 7apple0408086ｦ003 [56, 57]. 070000 CUL7 (KIAA0076, 010402020900 7), 6 50ｸ8090ｸ86 56ｦ00ｸ90ｸ70906 0ｹ10307 apple00 010700 096ｦ0 0702006 56ｦ0000900, 0ｸ802 016ｦ0apple0309apple076 56ｦ000 09 09 0000030902090ｷ309apple076 56ｦ000 6 5 2005 6 7. 006ｦ0 03apple07030706070300 6p21.1 [54]. ｨｦ00 06070603070903 0909 26 0ｹ101090700076 5 09 0107 09apple040003 00020701 CUL7, 01070307apple0ｸ806 0703000706090306fl 01 0600030006-06036 504 010402020900076 5, 0607060307fl0ｸ7000304 0909 0603apple04010304apple0007 066 5fl096ｦ000-000ｸ803 000201076 5, 6 5076 502000ｹ00000000ｸ803 6 5 08apple070ｷ30006060ｸ8 01020003070ｹ0000706 apple006 70402fl0ｷ30909, 03apple6ｦ00006 04010ｷ30909 06096 7006ｦ0026ｦ0, 0109060207apple07 000706 apple00-6 70402fl0ｷ30909 09 apple00086ｦ0apple6ｦ00ｷ30909 050401. 05. Huber et al [54] 070809-066ｦ00209 25 apple6ｦ00902090ｹ0000ｸ803 0304036ｦ00ｷ30906 02fl 6ｦ00000076 707 6 700006ｦ0, 19 0909 01070307apple0ｸ803 ｨC 08apple00 06016ｦ0090ｸ86 56ｦ000036ｦ0fl 08apple0008 006 5apple00030000006ｦ0fl 0300apple0309006ｦ00ｷ309fl 03apple6ｦ0000602fl0ｷ30909 09 6 ｨC 03090606000006-0304036ｦ00ｷ30906, 04 070200000ｸ803 06 3-02 060900 apple07030703 0909 29 0600030006 ｨC 6 50ｸ80307 0ｷ3006 5 0909 ｨｪ040009066ｦ0, 026ｦ0apple07010107, 06apple6ｦ0000ｷ30909, 02020809apple6ｦ0, 0509apple0909, ｨｨ07apple03046 76ｦ0020909, 0700apple036ｦ0000909, ｨｰapple09-006ｦ0000109, ｨｪ04apple0ｷ30909, 0700apple- 036ｦ0000909, 026 50603apple0909, ｨ 036ｦ0020909, 0504apple09006ｦ0036ｦ0, ｨ 00 0909 09 03apple6ｦ0-0909020909. 05 22 06000300fl03 070200000ｸ800 0ｸ80209 066 5fl096ｦ0000ｸ8 030008 04 0607 0706 01apple076 5000ｸ803 apple07 06036 50703. ｨｪ6ｦ0010903 07 apple6ｦ0090703, 04 fl01040306010903 070200000ｸ803 c0900 apple0703 3-02 6 50ｸ8090ｸ86 56ｦ0000306fl 00 090006036 50000000706, 0000 070809066ｦ000000706 apple6ｦ0000000 0304036ｦ0-0ｷ3090006 6 5 6 7000000 CUL7. 050ｸ8fl6 5020000006ｦ0fl 00 090006036 50000006ｦ0fl 0304036ｦ00ｷ309fl 6 5 6 7000000 CUL7 04 6 5060003 070200000ｸ803 09 6 50ｸ8fl6 5020000000ｸ806 6 76ｦ0080207030908 04016ｦ0090ｸ86 56ｦ00ｸ903 006ｦ0 0ｹ10202000103 070600076 56ｦ0030002fl. ｨｺ6ｦ00608apple070603apple6ｦ0000000-0007060300 fl0104030601076 707 060900 apple07036ｦ0 0009090107apple07060207060309 (ｨｹ0504) 0607-06036ｦ06 502fl0003 1 : 8775, 090209 10 006ｦ0 100 030ｸ806. 006ｦ0060002000009fl [58]. ｨ 02010004ｨｮ0004ｨ 00 050ｸ8 0002000007 08fl0300 fl01040306010903 006ｦ0060200 06036 50000000ｸ803 07020009-000006 (ｨｹ0403) 0807 6 50403 01apple090300apple09fl03. 0507-0800apple6 50ｸ803, apple6ｦ00608apple07-0603apple6ｦ00000000007060300 0903 09006ｦ00ｹ009030002000007 6 50ｸ80400, 0ｹ00003 6 5 0309apple076 50703 006ｦ0apple07 07006ｦ006000200000909, 6ｦ0 6 56ｦ0 096ｦ0 0702006 56ｦ00009fl ｨC 06080900070ｷ300apple00-000202flapple006ｦ0fl 6ｦ0036ｦ0010609fl I 0309086ｦ0 09 0ｹ10009090307080000090ｹ00006016ｦ0fl 030003-6 70003076 70207 0900000309fl ｨC 070300000600000ｸ8 01 0309apple076 50ｸ803 070ｹ06ｦ06 76ｦ003 006ｦ0-01070802000009fl 006ｦ0060200 06036 50000000ｸ803 07020009000006. 0507-6 50307apple0ｸ803, 02fl 016ｦ008 076 707 0909 000903 6 50ｸ8fl6 50200000ｸ8 070607 00000007060309 030702000104-02flapple000706 08apple09apple07 0ｸ8, 070302090ｹ06ｦ00ｸ90ｸ70900 0903 0703 060307 000ｸ803 02000007-030908076 5 6 5 apple046 70903 0807080402fl0ｷ309fl03, 6 5 01070307apple0ｸ803 070009 6 50603apple000ｹ06ｦ0-0ｸ90306fl 09006ｦ00ｹ009030002000007 apple000800. 046ｦ0 070600076 56ｦ0000909 apple0009040200036ｦ003076 5 6 700000003090107-0ｹ10809 0003090702076 7090ｹ00006010903 0906060200 076 56ｦ0000906 09 006ｦ0-010708020000000706 6 5 ｨｺ00060804 0209016ｦ00006010703 6 700000003090ｹ00006010703 apple006 709-0603apple00 (ｨｹ0104030601) 09000207apple036ｦ00ｷ30909, 6 507090307080007 000ｸ700 00000601070200-010903 ｰ016ｦ000 09 6ｦ003076 5 ｱ 006ｦ0 ｨｹ0403: 060900 apple0703 006ｦ0000909036ｦ0 06 0604-6ｦ003apple0702090006 09apple090300020000076 707 0000apple6 56ｦ0 09 080002006 700apple076 506010706 6ｦ00007036ｦ002090006 02000601070ｷ30903076 5 [59], 070200090000 06apple09 apple0006036ｦ0 09 060900 apple0703 030901apple07030909 06 010700 040103096 5000706 6 702040307030706 [15]. ｨｪ0007apple0003090ｹ00006016ｦ0fl 6 56ｦ0080007060300 03070200010402flapple0007-6 700000003090ｹ0000601076 707 0909040ｹ0000009fl 0ｹ10300070608000ｷ30902090ｹ00006010706 006ｦ0060200 06036 50000-000706 086ｦ0030702076 70909 0ｹ00002076 500016ｦ0, 006ｦ0applefl 04 06 03apple6ｦ0 090ｷ30907000007 07 07-09006ｦ00ｹ00000000ｸ80309 07 026ｦ00603fl0309 0906060200 076 56ｦ0000906 ｨC 6 700000003090ｹ000-06016ｦ0fl 6 7000300apple076 700000007060300 09 01020900090ｹ00006010906 080702090307apple02090903 07020009000006, 6 700000003090ｹ00006010700 apple6ｦ009000707 apple6ｦ0090900 0807080402fl0ｷ30906 0ｹ00002076 500016ｦ0, 0403070ｹ00000000900 026ｦ0010307apple076 5 0807080402fl0ｷ3090700000706 09-006ｦ003090109, 0708apple00 0002fl0ｸ90ｸ70903 006ｦ00107080200000900 0403, ｨC 096ｦ001020ｸ90ｹ06ｦ0-000306fl 000ｸ700 09 6 5 030703, 0ｹ00307 0807 07 000ｸ800 0906060200 076 56ｦ00009fl 6ｦ00ｸ903 09000603apple0403000003 01 0609060300036ｦ003090100 076 7apple070300076 707 apple6ｦ009000707 apple6ｦ00909fl 006ｦ0060200 06036 50000000706 086ｦ0030702076 70909 ｨC 0403070ｹ0000000090ｸ9 06040ｸ70006036 504-0ｸ90ｸ70903 09 060709 6ｦ000090ｸ9 036ｦ001 000007 0307 09030ｸ803 01020900090ｷ30906036ｦ003 00076 50ｸ803 01026ｦ00606090209016ｦ00ｷ30906 07020009000006. ｨｬ030006030007 096ｦ0030003090300, 0ｹ00307 08apple09 02090800000900 01 03070ｹ000070304 09006ｦ000090ｸ9 0300036ｦ000090903076 5 08apple07fl6 502000009fl 0304036ｦ0000300076 707 6 700006ｦ0 6 5 0200000703090800 030000 0002006 5-
1304020500000505ｨｪ050004040100 03ｨｦ0000ｨ 04ｨ ｨｬ ｨｹ01ｨｬｨｪｨｦ05 1313 0601076 707 096ｦ0 0702006 56ｦ00009fl 0807 06016ｦ0090ｸ86 56ｦ00003 07020000 0ｹ10202000103096 5-000ｸ800 0608070607 0ｸ8 09 000003090209016ｦ00ｷ30909 6 70000076 5-016ｦ000 09 6ｦ003076 5 030402000309026ｦ0010307apple096ｦ00200000ｸ803 096ｦ0 0702006 56ｦ0000906 ｨC 070200046ｦ0fl 0ｹ06ｦ0060300 006ｦ0040903 09006ｦ0000906 6 5 07 026ｦ0060309 03070200010402flapple000ｸ803 0300036ｦ0-00090903076 5, 0200086ｦ00ｸ70903 6 5 070600076 500 0409apple070107 apple6ｦ00608apple070603apple6ｦ0000000-000ｸ803 096ｦ0 0702006 56ｦ0000906, 00060300 09006ｦ00009fl, 080702040ｹ00000000ｸ800 08apple09 0909040ｹ000000909 apple00 010903 006ｦ0060200 0400030ｸ803 0207apple03 086ｦ0030702076 70909 [60]. 05ｨｨｨ 05ｨｦ01 00ｨ ｨｪ00ｨｺ02ｨｪｨｬｨｺ01 1. 07000007020700 09 6 70000076 700076 7apple6ｦ00209fl 006ｦ0apple07 07006ｦ0060002000009fl / ｨｨ07 apple00. 04.07. ｨｺ0ｸ80ｹ001076 56ｦ0. ｨｪ. 1. 07000007020700 006ｦ0060002000009fl ｨｺ0706060909 09 060708apple00 000200000ｸ803 0603apple6ｦ000. 05ｨｨ.: 046ｦ004016ｦ0, 2000. 611 06. 2. McKusick V.A. Genomics: structural and functional studies of genomes. // Genomics. 1997. V. 45. P. 244ｨC249. 3. 03070ｹ001076 5 04.ｨｨ., ｨｪ0903070200006 5-ｨｺ0006076 506010906 04.05. 0700076 7apple6ｦ002090ｹ00006016ｦ0fl 06apple00 6ｦ0 09 006ｦ0060200 06036 50000006ｦ0fl 086ｦ0030702076 709fl 0ｹ00002076 500-016ｦ0 // 0200 090ｷ3090006016ｦ0fl 6 700076 7apple6ｦ00209fl: 046ｦ0040ｹ000. 03apple. ｨｦ 000900-0601076 707 0703 00026ｦ0 07ｨｦ 050505ｨｺ. ｨｦ 0009000601, 1968. 05. 13ｨC18. 4. ｨｴ02apple070903060700 05.ｨｨ., 016ｦ002030ｸ801076 56ｦ0 00.07. ｨｬ0608000309 00006 5apple07-02076 7090ｹ00006010706 09 08060903096ｦ003apple090ｹ00006010706 6 700000003090109 // 070000000309-016ｦ0 08060903090ｹ00006010903 07020009000006. 02. 1970. 05. 186ｨC291. 5. 03070ｹ001076 5 04.ｨｨ., 0200026ｦ002076 56ｦ0 ｨｪ.05., 076ｦ0apple00016ｦ06 50ｷ3006 56ｦ0 ｨｺ.06. 09 apple. 0200 090107-6 700000003090ｹ00006010700 0909040ｹ000000900 006ｦ0060002000009fl ｨ 6ｦ0-086ｦ0 00076 707 ｨｨ6ｦ00309apple6ｦ0. I. 0507070300070400000900 080702076 5, 006ｦ0060200-06036 50000000ｸ800 0000apple6 50007-08060903090ｹ00006010900 096ｦ0 0702006 56ｦ00009fl 09 07-0ｸ70900 6ｦ00003apple0708076 700000003090ｹ00006010900 08apple0909006ｦ00109 // 070000000309016ｦ0. 1971. ｨｪ. 7. 6ｧ8 5. 05. 149ｨC159. 6. 0709000300apple 00.01. ｨｨ07080402fl0ｷ3090700006ｦ0fl 6 70000000309016ｦ0 09 0300 090ｷ309006ｦ0 // 05000603000901 ｨｺ020204. 2001. 6ｧ8 10. 05. 25ｨC31. 7. 046ｦ0060200 06036 50000000ｸ800 070200090009 6 5 0807080402fl0ｷ309fl03 0ｹ00002076 500016ｦ0 / ｨｨ07 apple00. 00.01. 0709000300apple6ｦ0. 02.: 0200 090ｷ309006ｦ0, 2002. 304 06. 8. ｨ 09000ｹ000000107 ｨｺ.02., 0002000ｹ00900076 56ｦ0 07.ｨ., 076ｦ0020109006ｦ0 05.02. 09 apple. 0509020200apple00000ｷ3096ｦ00ｷ309fl 0ｹ10300090ｹ00006010903 6 7apple040808 ｨｺ0706060909 0807 6 700-006ｦ003 006ｦ0060200 06036 50000000ｸ803 07020009000006 // 0200. 6 70000000309016ｦ0. 2007. ｨｪ. 6. 6ｧ82 (56). 05. 29ｨC37. 9. ｨｨ04090ｸ8apple006 5 05.ｨｨ. ｨ 09040ｹ000000900 070607 0000000706030006 apple6ｦ00608apple070603apple6ｦ0-00000009fl 006ｦ0060200 06036 50000000ｸ803 07020009000006 6 5 ｨ 6ｦ0086ｦ0 000706 0509 09apple09 // 0500060300. ｨｺ020204. 1984. 6ｧ8 7. 05. 76ｨC78. 10. ｨｨ04090ｸ8apple006 5 05.ｨｨ., ｨｴapple 0ｸ80000006 56ｦ0 00.05., 046ｦ0096ｦ0apple00000107 00.ｨｨ. 0700-000003090107-0ｹ10809 0003090702076 7090ｹ00006010700 0906060200 076 56ｦ0000900 006ｦ006000200-0009fl ｨｪ046 50ｸ8. ｨｪ07030601: STT, 1999. 256 06. 11. 04076 7076 5090ｷ30ｸ8006ｦ0 02.04., 026ｦ001060903076 56ｦ0 04.ｨｺ., 076ｦ000 0ｸ8 02.05., 020200010600006 56ｦ0 05.ｨｨ. 046ｦ0060200 06036 50000006ｦ0fl 086ｦ0030702076 709fl 0600030006, 07 apple6ｦ003096 504090306fl 6 5 020701 046ｦ00ｷ30907006ｦ0020000076 707 09000003apple6ｦ0 0200-090ｷ309000ｸ8 ｨｺ00060804 02090109 056ｦ0036ｦ0 (ｨｹ01040309fl) 06 1990 0807 1998 6 707 // 056ｦ0020000006 5070603. 0300. 0804apple00. 1999. 6ｧ8 1. 05. 26ｨC30. 12. ｨｨ04090ｸ8apple006 5 05.ｨｨ., 046ｦ0096ｦ0apple00000107 00.ｨｨ. 0700000003090107-0ｹ10809 00030907-02076 7090ｹ00006010700 0906060200 076 56ｦ0000900 006ｦ0060200 06036 50000000706 086ｦ0030702076 70909 6 5 ｨ 6ｦ0086ｦ0 000706 0509 09apple09. ｨｪ07030601: STT, 2000. 187 06. 13. 046ｦ0096ｦ0apple00000107 00.ｨｨ., 04076 7076 5090ｷ30ｸ8006ｦ0 02.04., 056ｦ0020ｸ90107-6 56ｦ0 ｨｦ.02., 06090209030700076 56ｦ0 02.04. ｨｺ6ｦ00608apple070603apple6ｦ00000000007060300 030700076 70000000ｸ803 006ｦ0060200 06036 50000000ｸ803 07020009000006 06apple00 09 06000200-0601076 707 006ｦ0060002000009fl ｨｺ00060804 02090109 056ｦ0036ｦ0(ｨｹ01040309fl) // 0200 09-0107-6 700000003090ｹ00006010700 0906060200 076 56ｦ0000900 006ｦ0060002000009fl ｨｺ00060804-02090109 056ｦ0036ｦ0(ｨｹ01040309fl): 05.006ｦ0040ｹ000.03apple. / ｨｺ00. 05.ｨｨ. ｨｨ04090ｸ8apple006 5 ｨｹ0104030601: ｨ 09-6 507 ｨｹ06 05ｨｦ ｨｺ0204, 2002. 05. 92ｨC114. 14. 046ｦ0096ｦ0apple00000107 00.ｨｨ., 056ｦ0080000076 56ｦ0 00.02., 046ｦ0096ｦ0apple00000107 05.02.09 apple. 02006ｦ0020909 0304036ｦ00ｷ30906 6 700006ｦ0 0ｹ10009090307080000090ｹ00006010706 006ｦ0-060200 06036 50000000706 0300036 70003076 70207 090000030909 6 5 fl01040306010706 0807-080402fl0ｷ30909 // 0200 090107-6 700000003090ｹ00006010700 0906060200 076 56ｦ0000900 006ｦ0-060002000009fl ｨｺ00060804 02090109 056ｦ0036ｦ0(ｨｹ01040309fl): 05.006ｦ0040ｹ000.03apple. / ｨｺ00. 05.ｨｨ. ｨｨ04090ｸ8apple006 5 ｨｹ0104030601: ｨ 09-6 507 ｨｹ06 05ｨｦ ｨｺ0204, 2002. 05. 151ｨC159. 15. ｨｪ6ｦ0apple06016ｦ0fl 00.02., ｨ 09000ｹ000000107 ｨｺ.02., 0002000ｹ00900076 56ｦ0 07.ｨ. 09 apple. 0503apple04010304apple6ｦ0 09 apple6ｦ009000707 apple6ｦ0090900 006ｦ0060200 06036 50000000706 086ｦ003070207-6 70909 6 5 ｨｺ00060804 02090100 056ｦ0036ｦ0(ｨｹ01040309fl) // 070000000309016ｦ0, 2004. ｨｪ. 40. 6ｧ8 11. 05. 1530ｨC1539. 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