- PDB-6lhv: Structure of FANCA and FANCG Complex -
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基本情報
登録情報
データベース: PDB / ID: 6lhv
タイトル
Structure of FANCA and FANCG Complex
要素
Fanconi anemia complementation group A
Fanconi anemia complementation group G
キーワード
DNA REPAIR / nuclear localization / fanconi anemia core protein / fanconi anemia complementation group a / fanconi anemia complementation group g / interstrand crosslink repair
ジャーナル: Nucleic Acids Res / 年: 2020 タイトル: Structural basis of the fanconi anemia-associated mutations within the FANCA and FANCG complex. 著者: Eunyoung Jeong / Seong-Gyu Lee / Hyun-Suk Kim / Jihyeon Yang / Jinwoo Shin / Youngran Kim / Jihan Kim / Orlando D Schärer / Youngjin Kim / Jung-Eun Yeo / Ho Min Kim / Yunje Cho / 要旨: Monoubiquitination of the Fanconi anemia complementation group D2 (FANCD2) protein by the FA core ubiquitin ligase complex is the central event in the FA pathway. FANCA and FANCG play major roles in ...Monoubiquitination of the Fanconi anemia complementation group D2 (FANCD2) protein by the FA core ubiquitin ligase complex is the central event in the FA pathway. FANCA and FANCG play major roles in the nuclear localization of the FA core complex. Mutations of these two genes are the most frequently observed genetic alterations in FA patients, and most point mutations in FANCA are clustered in the C-terminal domain (CTD). To understand the basis of the FA-associated FANCA mutations, we determined the cryo-electron microscopy (EM) structures of Xenopus laevis FANCA alone at 3.35 Å and 3.46 Å resolution and two distinct FANCA-FANCG complexes at 4.59 and 4.84 Å resolution, respectively. The FANCA CTD adopts an arc-shaped solenoid structure that forms a pseudo-symmetric dimer through its outer surface. FA- and cancer-associated point mutations are widely distributed over the CTD. The two different complex structures capture independent interactions of FANCG with either FANCA C-terminal HEAT repeats, or the N-terminal region. We show that mutations that disturb either of these two interactions prevent the nuclear localization of FANCA, thereby leading to an FA pathway defect. The structure provides insights into the function of FANCA CTD, and provides a framework for understanding FA- and cancer-associated mutations.
The authors know the complete sequence as follows. But they are not sure which part of sequential ...The authors know the complete sequence as follows. But they are not sure which part of sequential amino acids corresponds with sample sequence. So the number of residues is meaningless. chain C sequence MDYKDDDDKDYKDDDDKGSGGSGMAGDCLTLWMEENNVIVNQWRDSASYANTFTEKQKLQLLHADLYQLLQKIQGLPATPQSLPLELSVLYNMLIFHIHST SGFQDEHNKHIAETLSRVLRVCSPGVNVQSNEELWHQLVHGGYSSELAASLHRLAGLQGALWLAEEKLQEVHDLFSLLLHPEDSECSTNLRAEEKLLALLK AWRVSNEAEEKVLVVQPAQQLRDVLYTSAAFLQGILAMEASQHPYAINLFGEAAESLCSGRVLAQVYTCLGFCFQKMEKPQTALQYWKQALHLDFQCLAAL YHSSSVYKEMGNVDAELEALNLLYKALLSPEQEAPSTKTCFLIGTEQIVNAPSLTSAVRAPSPWKVKYLIARRCLENKRGEEAVEHYLDLLALLQDESHQD LLPNPEPLIRIPEVFLETAAGLVLEKRYQDAMTVCEEVLGRTDDLIPGSLSVDSTQSKSNVMAEQLNLVLWASAAHLHQGLSHGLLGDQKESVTEYTKCIN LLVKVQFIDTGCASPHCSRGSGDEMEISLIGLCSKEETVLCALKAAAFLGRSRQFLALGKHKESLLNMQLSLQASPAFPGAALTLVGHLIKLGRRTEAGSY LKRYQSERKTLADQWEAMKRNLPLYLDPHINHTFPAEDSLIKELEESAEEMQSEAVV chain B,A sequence MWSHPQFEKGSGGSGMSAVSGFTPSGQKRSLAELLDGRVKRLDRKSNNSVLQEAALYLLSCNQDVSQFLSEVEAPPYKKTCNPENPVSIKSRMPSAAFVGS TLKDQASCLKISPGILTAKAAVANIQQICQACGDSSAVLNPEQREKLCSLLKTLKILLAENCFCRSLFCKEIWIHRPPLVFEAVWHLHNEGIVCLDEILES CKDTISAVDWLFSEMCSLCLYIDNSSLAGDLAEKMISDFQALLVENSFRRSSATERILEQHKTEEICLSILDKLLSWLLDAVSVEKSDKSSAEQHWLSAFE VHRYRARVVPESIEQFFIHTLTQVLTFKPKLKVSDAIQCQANWSFVKTSTLLTDLYRKLFVALSAEKLIAHIQLVLDTQEVNWHHVLTCVSCLVICLPEAQ QLIKDLLCRLLTHAFESYELEGMITAFLIVRQAALEGPAAFVSYTEWFKCTFGAANSYHGNSKKSLVFLLKFLSDIVPFEAPQYLKVHVLHPPFVPTKYRP LLMEYISLAKTRLTDMKVSIEDMGLYEDLSARSNKVQPESQAHQDVEKALNIFENTGKIPASVMEASIFRRPYFTSRFLPALLTPRVLPAAPDALMLLIDS MKRADKIPTNMFNAYIEACEQEKLRKQKGRQQMDQSLPDEPLGILQSALSDLRPLVTDANKYEDVSAQVAVISEKLIAVMGEQKVDDDQVAAKFLKLEDGA QLDIQEQTVADLLLTCFCQCLIAASGTNPPDRQGQWPTLYVKMLCGHQWAFAAVLRRMLQLLRFQAPFLKDSHIVGLAAFSIHLHECQPSLQFLITGVQNL EHYWENLLNLLCSDSVGVCLKLCTAAISYAFCRFSELHQDIFSGCVPPLFLRKLQYLVPRLIWETRGEVIRDDEEADSPLNWNLYALAGWKEAALSLWNQN RLQGLLREKSFQVTFMDWLLWEMTLKSNNDVLCDTDRQEYQRWAVNHYLSESSVVGGCNGDLERGCITIAEAVLQFSNRHIQHSEWESRNISMLKSHTGLG DILCRLQELICDIVTSHHQKGRRHFFFAIFYQRLELHKGKKELSNHLSKQGVLEMCCRILLGLPPLFLINTPSEKGIRTLGSEDFWQFVNKELKNLGPRGY ALPYNITAHFFRGVISASVQCKDSSEAVNSILSATYSTCPALLISAAVGWPQLDPVLRSQWCSLFGVDLPKELRTLREQQASVDSCLSQGEKLSLSCTPWL SAAFLYSTVQRKKLPCSRMLEILDGLSSNFSMVLISLLFFSVMDIIYMFLKDGRKHKDLLENCVHIIHCLEQKGETWVWLFQMTDERKPELGLHLHRAASD VFLNLMPFAFFWLVPSLQLEQVVQQQDFLVIALDMYHKFLQLFVDGSPLSSLSAKSHHLDSHDVFTCGRQFLLCCVPKCQKPNSAILKKMLESWEEHDPEL AAVLTRSFKAPDDYDDLFFEPVF
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実験情報
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実験
実験
手法: 電子顕微鏡法
EM実験
試料の集合状態: PARTICLE / 3次元再構成法: 単粒子再構成法
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試料調製
構成要素
名称: FANCA / タイプ: COMPLEX / 詳細: homo dimer / Entity ID: all / 由来: RECOMBINANT
分子量
値: 500 kDa/nm / 実験値: YES
由来(天然)
生物種: Xenopus laevis (アフリカツメガエル)
由来(組換発現)
生物種: Trichoplusia ni (イラクサキンウワバ)
緩衝液
pH: 8
試料
濃度: 0.8 mg/ml / 包埋: NO / シャドウイング: NO / 染色: NO / 凍結: YES