Makalah Mtokondria

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    CDC F

    QNJMDE^B^DJ

    D* Bd}ds Cnbdkdjo

    \nb dmdbde gfjfd}~s knefm~qdj d}d~ gnjvns~qdf kdwd|dj fjm~|}sf vdjo dgd}

    knlfb" mf mdbdgjvd }nsmdqd} isodjnb'isodjnb vdjo gfsfq qdcsfk'qdcsfk kfgfd |nb vdjo

    gnjoibde mdj gnjmf|}sfc~|fkdj ed|fbjvd qdmd cdofdj vdjo gngc~}~ekdjjvd* Gd|fjo'

    gd|fjo isodjnbd gngq~jvdf qnsdj vdjo cnscnmd'cnmd mdbdg }~c~e" }n}dqf dj}dsd isodjnbd

    |d}~ mnjodj isodjnbd vdjo bdfj gd|fe |dbfjo cnse~c~jodj* Mf mdbdg |f}iqbd|gd }nsmdqd}

    isodjnb gngcsdj bdfj vdjo cns}fjmdk |ncdodf mdq~s |nb vdf}~ gf}ikijmsfd* Jdgd

    gf}ikijmsfd mfcnsfkdj ibne |nisdjo qdkds cnsjdgd Cnjmd %456?'4565&* Isodjnbd vdjo

    cnj}~kjvd gngdjhdjo fjf mfh~gqdf qdmd edgqfs |ng~d |nb n~kdsfi}fk* Gf}ikijmsfd

    mfcd}d|f ibne gngcsdj sdjokdq" gngcsdj mdbdg gnjodmdkdj qnsb~d|dj kn mdbdg gd}sfk|

    mnjodj gngcnj}~k qnjijhibdj'qnjijhibdj vdjo mf|nc~} ksf|}d* Gngcsdj gf}ikijmsfd

    mf|~|~j ibne bfqiqsi}nfj" bncfe }fqf| mdsf gngcsdj qbd|gd* _dbd~q~j gf}ikijmsfd

    mfc~jok~| ibne gngcsdj" gf}ikijmsfd c~kdjbde cdofdj mdsf |f|}ng njmigngcsdj* Qsi}nfj

    gngcsdjjvd }fmdk mfc~d} ibne SN" }n}dqf ibne sfci|ig cncd| mdbdg |f}i|ib mdj ibne

    sfci|ig vdjo mfkdjm~jo mdbdg gf}ikijmsfd f}~ |njmfsf* Isodjnbd fjf }fmdk |dhd gngfbfkf

    sfci|ig" }n}dqf h~od gnjodjm~jo MJD mdbdg h~gbde |nmfkf}* MJD fjfbde vdjo

    gngqsiosdg |fj}n|f| qsi}nfj vdjo mfc~d}mdbdg sfci|ij gf}ikijmsfd* Gf}ikijmsfd h~od

    gngq~jvdf `~jo|f mdbdg ik|fmd|f" mnefmsionjd|f" `i|`isfbd|f ik|fmd}f` mdj sn|qfsd|f |nb*

    \}s~k}~s mdj |f|}ng njxfgjvd mf|n|~dfkdj mnjodj kndjnkdsdodgdj `~jo|fjvd* Mdsf ~sdfdj

    mfd}d| qngdkdbde }ns}dsfk ~j}~k gngcded| gf}ikijmsfd mdj edb'edb vdjo cnsknjddj

    mnjodj ~jo|fjvd*

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    C* S~g~|dj gd|dbde

    Mdsf bd}ds cnbdkdjo mfd}d| mdqd} mfdgcfb cncnsdqd s~g~|dj gd|dbde |ncdodf

    cnsfk~}8

    Cdodfgdjd |}s~k}~s mdj `~jo|f mdsf gf}ikijmsfd9

    Cdodfgdjd gnkdjf|gn sn|qfsd|f |nb mdsf gf}ikijmsfd9

    L* ]~h~dj

    ]~h~dj mdsf s~g~|dj gd|dbde mfd}d| dmdbde8

    ^j}~k gnjon}de~f |}s~k}~s mdj `~jo|f mdsf gf}ikijmsfd*

    ^j}~k gnjon}de~f gnkdjf|gn sn|qfsd|f |nb

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    CDC FF

    QNGCDED\DJ

    D* \}s~k}~s mdj @~jo|f Gf}ikijmsfd

    Gf}ikijmsfd cnsd|db mdsf kd}d V~jdjf Gf}i vdjo cnsds}f cnjdjo" mdj leijmsfij

    vdjo cnsds}f |nqns}f osdj~b%c~}fs'c~}fsdj&" |nefjood mdqd} mfds}fkdj |ncdodf isodjnbd mnjodj

    sdjokdfdj c~}fs'c~}fs vdjo |nqns}f cnjdjo*

    Gf}ikijmsfd dmdbde isodjnb |nb n~kdsfi} vdjo cns`~jo|f |ncdodf isodj sn|qfsd|f

    qngcdjokf} njnsof mnjodj gnjoed|fbkdj dmnji|fj }sfqei|qed} %D]Q&* H~gbde gf}ikijmsfd

    }fdq |nb }nsodj}~jo hnjf| |nb mdj isodjf|gn* Gf}ikijmsfd mf}ng~kdj mdbdg h~gbde cdjvdk

    qdmd |nb vdjo dk}fpf}d| gn}dcibf|gnjvd }fjoof vdf}~ |nb'|nb kij}sdk}fb |nqns}f |qnsgd qdmd

    cdofdj nkisjvd" |nb i}i} hdj}~jo" mdj |nb vdjo dk}f` gngcnbde |nqns}f nqf}nbf~g" dkds

    sdgc~}" mdj nqfmnsgf| k~bf}*

    Gf}ikijmsfd mfm~od cnsd|db mdsf cdk}nsf |ns~qd Sflkn}}|fd vdjo efm~q cncd|"

    kng~mfdj mf}nbdj jnjnk'givdjo |nb n~kdsfi} mdj gngcnj}~k njmi|fgcfi|f| |d}~ |n}njode

    gfbfds }de~j bdb~*

    Gf}ikijmsfd gns~qdkdj sdjokdfdj isodjnbd vdjo ~jfk kdsnjd gngfbfkf MJD

    }ns|njmfsf vdjo mf|nc~} MJD gf}ikijmsfd mnjodj |f`d}'|f`d} vdjo |qn|f`fk* MJD

    gf}ikijmsfd gngfbfkf lfsf'lfsf vdjo cnscnmd mdsf MJD j~kbn~| mf}fjhd~ mdsf ~k~sdj" h~gbde

    onj" mdj cnj}~k*

    Cn|ds onjig qdmd MJD gf}ikijmsfd snbd}f` knlfb dqdcfbd mfcdjmfjokdj mnjodj

    onjig MJD qdmd j~kbn~|* ^k~sdj onjig MJD gf}ikijmsfd qdmd }fdq'}fdq isodjf|gn

    |djod}bde cnspdsfd|f* Qdmd gdj~|fd ~k~sdj MJD gf}ikijmsfd dmdbde 40"0 kc" |nmdjokdj

    qdmd Msi|iqefbd gnbdjiod|}ns k~sdjo bncfe 45"= kc* Qdmd kedgfs" ~k~sdj onjig snbd}f`

    bncfe cn|ds vdf}~ 5= kc*

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    ]fmdk |nqns}f MJD j~kbn~| vdjo cnscnj}~k bfjnds" g}MJD cnscnj}~k bfjokdsdj*

    \ncdofdj cn|ds g}MJD gngcdwd onjn vdjo cns`~jo|f mdbdg qsi|n| sn|qfsd|f |nb*

    Nk|qnsfgnj vdjo mfbdk~kdj mnjodj gnjoefbdjokdj g}MJD qdmd \* lnsnpf|lndn

    gnj~jh~kdj qnj~s~jdj }fjokd} qns}~gc~edj vdjo |fojf fkdj vdjo mf}djmdf mnjodj

    gnjonlfbjvd ~k~sdj |nb*

    Gf}ikijmsfd gns~qdkdj isodjnbd vdjo cns~qd kdj}~jo vdjo mf|nbdq~}f ibne m~d gngcsdj"

    vdf}~ gngcsdj mdbdg mdj gngcsdj b~ds" |nefjood gf}ikijmsfd gngfbfkf m~d

    kigqds}ngnj" vdf}~ s~djo dj}ds gngcsdj %fj}nsgngcsdjn |qdln& mdj gd}sfk| %gd}sfu&

    gf}ikijmsfd vdjo mf|nbfg~}f bdjo|~jo ibne gngcsdj mdbdg*

    Gngcsdj b~ds

    Gngcsdj b~ds gnjodjm~jo qsi}nfj }sdj|qis} vdjo mf|nc~} qisfj* Qisfj

    gngcnj}~k |db~sdj vdjo cns~k~sdj snbd}f` bncfe cn|ds mf bdqf|dj odjmd bfqfm gngcsdj b~ds;

    |nefjood gngcsdj b~ds mdqd} mfdjoodq |ncdodf |dsfjodj vdjo gng~jokfjkdj bibi|jvd fij

    gd~q~j gibnk~b knlfb cns~k~sdj > kMd d}d~ k~sdjo" }nsgd|~k qsi}nfj cns~k~sdj knlfb*

    Gibnk~b'gibnk~b }ns|nc~} cncd| gngd|~kf s~djo dj}ds gngcsdj" jdg~j |ncdofdj cn|ds

    }fmdk gnbnwd}f gngcsdj mdbdg vdjo cns|f`d} fgqns'gndcnb* Fjf cnsds}f cdewd mdbdg edb

    kdjm~jodj gibnk~b knlfb mf s~djo dj}ds gngcsdj cns|f`d} nk~fpdbnj mnjodj |f}i|ib

    |nmdjokdj mf s~djo gd}sfk| cnscnmd* Qsi}nfj vdjo }nsbn}dk qdmd gngcsdj b~ds gnbfq~}f

    cnscdodf njxfg vdjo }nsbfcd} mdbdg cfi|fj}n|f| bfqfm gf}ikijmsfd mdj njxfg'njxfg vdjo

    gnjo~cde |~c|}sd} bfqfm gnjhdmf cnj}~k bdfj ~j}~k |nbdjh~}jvd mfgn}dcibf|gn mf gd}sfk|

    gf}ikijmsfd*

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    Gngcsdj mdbdg mdj ksf|}d

    Gngcsdj mdbdg mdj gd}sfk| gf}ikijmsfd }nskdf} nsd} mnjodj dk}fpf}d| ~}dgd

    gf}ikijmsfd vdf}~ }nsbfcd} mdbdg |fkb~| d|dg }sfkdscik|fbd}" ik|fmd|f d|dg bngdk mdj

    qngcnj}~kdj njnsof* Sdj}df sn|qfsd|f }nsmdqd} mdbdg gngcsdj mdbdg fjf* Gngcsdj mdbdg

    mdsf |nbfg~} gf}ikijmsfd |djod} cnscnbf}'cnbf} gns~dk kn cdofdj mdbdg gd}sfk mnjodj qibd

    |nqns}f }dc~jo d}d~ mnjodj qibd bfs bngcdsdj mf cnscdodf }ngqd}* \d}~ cnbf}dj mf|nc~} ksf|}d*

    Qdmd |ncdofdj cn|ds gf}ikijmsfd }~gc~edj ksf|}d }dc~jo bncds }nscnj}~k mnjod

    |ngq~sjd }dqf cnsdodg gnj~s~} hnjf| |nb" ~g~s" mdj }fjokd} qnskngcdjodj* \d}~ ksf|}d

    cnsodc~jo mnjodj ksf|}d bdfj mf cdofdj mdbdg gf}ikijmsfd gngcnj}~k wdmde

    fj}nsgngcsdj bfs'kdj}~jo vdjo cns|dgc~jodj dj}ds gnsnkd* Qdmd |nb bdfjjvd" }n}dq }nshdmf

    gimf`fkd|f bdfj* Dqdq~j cnj}~kjvd" ksf|}d gnjodjm~jo |ncdofdj cn|ds njxfg vdjo

    gnjokd}dbf|f| bdjokde qdmd |f|}ng qnjodjok~}dj nbnk}sij |n}nbde md~s ksnc| |nefjood

    qnjfjokd}dj b~d| qnsg~kddj |djod}bde qnj}fjo*

    S~djo dj}ds gngcsdj

    S~djo dj}ds gngcsdj dmdbde s~djo vdjo cnsdmd mf dj}dsd gngcsdj b~ds mdj

    gngcsdj mdbdg gf}ikijmsfd* S~djo fjf gnjodjm~jo |nkf}ds 0. mdsf }i}db qsi}nfj

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    gf}ikijmsfd mdj cncnsdqd njxfg vdjo cnknshd gnjoo~jdkdj D]Q %dmnji|fjn

    }sfqei|qed}n& vdjo }njode gnbnwd}f s~djo }ns|nc~} ~j}~k gng`i|`isfbd|f j~kbni}fmd bdfj*

    Gd}sfk|

    \ncdofdj cn|ds %|nkf}ds 0?.& qsi}nfj gf}ikijmsfd mfh~gqdf qdmd cdofdj gd}sfk|*

    Njxfg'njxfg vdjo mfc~}~ekdj ~j}~k qsi|n| ik|fmd|f qfs~pd}" d|dg bngdk mdj ~j}~k

    gnjhdbdjkdj |fkb~| d|dg }sfkdscik|fbd} }nsmdqd} qdmd gd}sfk| fjf*

    ]nsmdqd} qdmd Njxfg

    Gngcsdj B~ds

    S~djo dj}ds gngcsdj

    Gngcsdj mdbdg

    Gd}sfk|

    JDME'|f}iksig L ik|fmisnm~k}d|n"

    |f}iksig c" d|fb KiD |fj}n}d|n" gijidgfj

    ik|fmd|d" kfj~snjfj efmsibd|d*Ksnd}fj kfjd|d" dmnjfbd} kfjd|d

    \f}iksig c" l" l4"d "d

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    C* Qsi|n| Sn|qfsd|f

    Sdj}df Sn|qfsd|f

    Sdj}df sn|qfsd|f mdj fjefcf}isjvd mdqd} mfbfed} |ncdodf sfjokd|dj hdb~s gn}dcibfk

    gf}ikijmsfd qdmd |ng~d kigqbnk| vdjo cnsdmd mf gngcsdj mdbdg mdj mdqd} mfldqdf ibne

    |~c|}sd} cdfk vdjo cnsdmd qdmd gngcsdj gd~q~j qdmd gd}sfk|* ]nbde mfkn}de~f q~bd

    cnscdodf fjefcf}is sdj}df sn|qfsd|f mdj n`nk kbfjfk bdfjjvd vdjo mdqd} mfdjoodq |ncdodf

    qnjon}de~dj dwdb mdsf gf}ileijmsfdb gnmflfjn* ^j}~k gng~mdekdj qsi|n| sn|qfsd|f |nb

    mfcnmdkdj gnjhdmf }fod cdofdj 8

    4* Obfkibf|f|

    3* \fkb~| Ksnc|

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    Qdmd cncnsdqd knhdmfdj ~j}~k gnbdjh~}kdj qngnldedj ob~ki|d qdmd |d}~ |f|}ng"

    knm~d JDME eds~| mfik|fmd|f" kdsnjd f}~ nbnk}sij mdj qsi}ij vdjo mfkdjm~jojvd eds~|

    mfcncd|kdj* Cfbd ik|fonj }ns|nmfd mdbdg |nb" nbnk}sij mdj qsi}ij mdqd} mfo~jdkdj ~j}~k

    gnjoed|fbkdj D]Q vdjo bncfe cdjvdk* Cfbd ik|fonj }fmdk }ns|nmfd mdbdg |nb" |nb dkdj

    gnjmdqd}kdj nbnk}sij mdj dkdj gngcnj}~k kngcdbf JDM gnbdb~f hdbdj vdjo bdfj* Knm~d

    hdbdj }ns|nc~} mfjdgdkdj `nsgnj}d|f* @nsgnj}d|f dmd ngqd} gdldg dj}dsd bdfj 8 `nsgnj}d|f

    djdnsic; `nsgnj}d|f dbkieib; mdj `nsgnj}d|f d|dg bdk}d}* @nsgnj}d|f dbkieib gnjoed|fbkdj

    LI3 mdj dbkieib" |nmdjokdj `nsgnj}d|f d|dg bdk}d} gnjoed|fbkdj d|dg bdk}d}*

    Qdmd hdbdj djdnsic vdjo knm~d" qfs~pd} vdjo mfed|fbkdj |nbdgd qsi|n| obfkibf|f|

    mf~cde gnjhdmf bdk}d}* @nsgnj}d|f bdk}d} mdqd} mfbfed} qdmd cdjvdk gfksiisodjf|gn mdj

    |nbdgd bd}fedj %ibdesdod& vdjo cnsd}" qdmd hdsfjodj i}i} kf}d* ^g~gjvd gn}dcibf|gn

    djdnsic cnse~c~jodj mnjodj cnj}~k knefm~qdj vdjo bncfe |nmnsedjd" }n}dqf knjvd}ddjjvd

    dmdbde cdewd |nbdgd knofd}dj vdjo cnsd}" i}i} kf}d gnjodjmdbkdj gnjmdqd}kdj D]Q

    |nbdgd obfkibf|f| mdj cns}~s~}'}~s~} gngcdjo~j |nh~gbde bdk}d} vdjo l~k~q cn|ds mdbdg

    }~c~e kf}d |njmfsf* _dbd~q~j kf}d gnjodgcfb ik|fonj mdj i}i} kf}d mdqd} gnjoo~jdkdj

    bncfe cdjvdk qsim~k D]Q gnbdb~f qsi|n| sn|qfsd|f ik|fmd}f` |nbdgd knofd}dj sfjodj"

    qnjvnmfddj ik|fonj }fmdk gngdmdf ~j}~k gngnbfedsd knc~}~edj D]Q vdjo cdjvdk*

    Kdsnjd f}~ kf}d eds~| gnjodjmdbkdj obfkibf|f| vdjo }fmdk gngc~}~ekdj ik|fonj " |nqns}f

    qdmd JDME vdjo mfik|fmd|f" mdj |nqns}f qdmd `nsgnj}d|f dbkieib" fjf mfo~jdkdj ~j}~k

    gngcnj}~k qsim~k dkefs vdjo mdbdg edb fjf dmdbde bdk}d} JDM vdjo cncd| mfo~jdkdj bdof

    qdmd obfkibf|f|* Bdk}d} eds~| mfcns|fekdj mdsf i}i} dods q~bfe kngcdbf* Qdmd }~c~e bdk}d}

    mfcdwd gnbdb~f dbfsdj mdsde kn ed}f" mfgdjd mf~cde gnjhdmf qfs~pd}* Cncnsdqd qfs~pd}

    |nsfjo mdqd} gnjngc~| pns|f hdbdj cdbfk mdsf obfkibf|f| mdj mnjodj qnjvfgqdjdj D]Q mdsf

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    hdbdj ik|fmd}f` mf~cde kngcdbf gnjhdmf ob~ki|d* Ob~ki|d kng~mfdj mf|fgqdj mdbdg ed}f

    d}d~ qdmd i}i} mdbdg cnj}~k obfkionj* Dqdcfbd qfs~pd} gd|~k qdmd hdbdj mfgdjd ik|fonj

    mfo~jdkdj gdkd mf|fjfbde |ncdofdj cn|ds njnsof cncd| vdjo cn|ds mdsf ob~ki|d dkefsjvd

    mfcncd|kdj ~j}~k gnbdk~kdj knshd*

    \fkb~| Ksnc|

    M|fjf qfs~pd} mdsf obfkibf|f| |nldsd bnjokdq mfik|fmd|f gnjhdmf LI* _dbd~q~j

    }fmdk cdjvdk D]Q vdjo }nscnj}~k edjvd m~d gibnk~b* Bdjokde dwdb md~s ksnc|

    gnjvdjok~} ik|fmd|f mdj efbdjojvd LI3 mdsf qfs~pd} mdj qnjoodc~jodj |f|d ~jf} d|n}d} 3

    kdscij mnjodj |njvdwd vdjo gnjodjm~jo cnbnsdjo vdkjf ki'njxfg gngcnj}~k d|n}fb

    LiD fjf mdj qnsdj bdfjjvd mfmd~s ksnc| gns~qdkdj dbd|dj qnj}fjo gnjodqd cnbnsdjo

    }nsgd|~k edsd n|nj|fdb*

    @~jo|f ~}dgd mdsf |fkb~| ksnc| dmdbde gnjvnmfdkdj nbnk}sij ~j}~k mf}sdj|qis}

    gnbdb~f |f|}ng }sdj|qis} nbnk}sij vdjo dkdj mfo~jdkdj njnsofjvd ~j}~k gnjmdqd}kdj

    qnscnmddj qi}nj|f kfgfd %Lengfi|gi}fl mf``nsnj}fd& mf gf}ikijmsfd* \ncnb~g nbnk}sij mdj

    qsi}ij gng~bdf qnshdbdjdjjvd" |ngnj}dsd mf|fgqdj qdmd m~d gibnk~b qnjnsfgd" JDM+

    mdj @DM" gnsnm~k|f gibnk~b fjf gnjhdmf JDME+ + E+ mdj @DME3* Md~s ksnc|

    gnbdk~kdj qnjodgcfbdj cncnsdqd nbnk}sij mdsf d|dg isodjfk mdj gnjodjok~} nbnk}sij

    }ns|nc~} kn JDM* Mdbdg md~s ksnc| }fmdk dmd njxfg mnefmsionjd|n" mdsf md~s fjf vdjo

    gnjoo~jdkdj JDMQ |ncdodf qnjnsfgd nbnk}sij* Cdekdj JDMQ cfd|djvd edgqfs }dk

    }nsmn}nk|f mdbdg gf}ikijmsfd }~gc~edj kndmddj vdjo cnsbdwdjdj mnjodj kbisiqbd| vdjo

    mdbdgjvd JDMQ |djod} cnsbfgqde" |nmdjokdj JDM bncfe |nmfkf}* ]fmdk edjvd JDME mdj

    ~cfk~fjib vdjo gns~qdkdj qsim~k qnj}fjo mdsf md~s ksnc|" }dqf 4 gibnk~b D]Q mfcnj}~k

    mdsf DMQ + Q |nbdg qnjo~cdedj |~k|fjd} kinjxfg D gnjhdmf d|dg |~k|fjd}*

    Qsi|n| fjf mfg~bdf kn}fkd qfs~pd} knefbdjodj |nc~de d}ig vdjo mfcncd|kdj

    |ncdodf LI3 vdjo bncfe qnj}fjo" qfs~pd} mfik|fmd|f* Kdsnjd f}~" |nqd|djo nbnk}sij vdjo bdfj

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    ]sdj|qis} Nbnk}sij

    \f|}ng qnjodjok~}dj nbnk}sij mdj `i|`isfbd|f JDME }nsmdqd} mf gf}ikijmsfd

    cnsd|db mdsf < qsi|n| ~}dgd ; md~s ksnc|" obfkibf|f| mdj mf md~s ik|fmd|f obf|fj vdjo

    mfed|fbkdj |nbdgd `i}isn|qfsd|f* Cfbd JDME mfik|fmd|f dkdj mfed|fbkdj D]Q* Mnjodj ldsd

    |ns~qd ~cfk~fjib mfed|fbdkdj ibne d|dg |~k|fjd} mnefmsionjd|n mdbdg md~s ksnc| h~od

    mfik|fmd|f ~j}~k gnjoed|fbkdj D]Q* _dbd~q~j ik|fmd|f fjf gnbfcd}kdj qnjodgcfbdj I3

    mdj qngcnj}~kd E3I cdfk JDME d}d~q~j ~cfk~fjib }fmdk cnsodc~jo |nldsd bdjo|~jo

    mnjodj I3 ~j}~k gngcnj}~k E3I* Vdjo }nshdmf" nbnk}sijjvd mf}sdj|`ns gnbdb~f cncnsdqd

    |njvdwd dj}dsd |ncnb~g E3I }nscnj}~k* Qngcdwd nbnk}sij fjf gns~qdkdj |f|}ng

    qnjodjok~} nbnk}sij gf}ikijmsfd* Qnjodjok~}dj nbnk}sij mfg~bdf mdsf qngcdwd vdjo

    }nsgimfjdgfkd |~bf} ~j}~k mfsnm~k|f kn qngcdwd vdjo gngq~jvdf knlnjmns~jodj vdjo

    bncfe cn|ds ~j}~k gnjnsfgd nbnk}sij*

    Qnjodjok~}dj nbnk}sij mfg~bdf mnjodj JDME+ + E+ vdjo }nscnj}~k mf gd}sfk|

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    Qngcnj}~kdj D]Q qdmd gf}ikijmsfd

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