6V4U: SMCR8-C9orf72-WDR41 complex

Cryo-EM structure of SMCR8-C9orf72-WDR41 complex. Determined by electron microscopy at 3.8 Å resolution. Released 2 Sept 2020.

Method
Electron microscopy
Resolution
3.8 Å
Organism
Homo sapiens
Chains
3
Atoms
7,074
Mol. weight
211.32 kDa
Released
2 Sept 2020

Explore 6V4U in 3D Show helices and sheets RCSB PDB PDBe

Secondary structure: helices and β-sheets

6V4U contains 31 α-helices and 44 β-strands across 3 chains. Residue ranges use author residue numbering, as in the PDB file, from PDBe. To see them in 3D, choose the Cartoon representation with Secondary structure coloring: helices, sheets and coils get different colors.

Chain A: 14 helices, 9 β-strands

ElementResiduesLengthSheet
β-strand27-33711
β-strand39-43511
α-helix56-6813
β-strand85-87312
β-strand92-94312
β-strand99111
β-strand109-115711
β-strand117112
α-helix118-1203
α-helix121-1244
α-helix128-14013
α-helix155-17016
α-helix198-20811
α-helix212-22312
β-strand238113
α-helix242-2487
β-strand285113
α-helix293-2997
α-helix378-38710
α-helix395-42026
α-helix425-43814
α-helix446-45813
α-helix465-4739
Chain B: 15 helices, 10 β-strands
ElementResiduesLengthSheet
β-strand60114
α-helix84-9714
β-strand122112
β-strand123115
β-strand136115
β-strand137114
β-strand155114
α-helix166-21651
α-helix227-25226
α-helix321-33919
α-helix344-35815
α-helix367-38115
α-helix707-7159
α-helix720-72910
β-strand732-737616
α-helix741-7455
α-helix748-7514
α-helix772-7787
β-strand784-786316
β-strand807-810416
β-strand815116
α-helix826-8316
α-helix843-86725
α-helix880-89112
α-helix901-91313
Chain C: 2 helices, 25 β-strands
ElementResiduesLengthSheet
β-strand37-3931
β-strand46-5052
β-strand58-6142
β-strand66-6723
β-strand6812
β-strand79-8023
β-strand105-10954
β-strand115-11954
β-strand127-12934
β-strand140-14125
β-strand148-14925
α-helix175-1762
β-strand180-18126
β-strand188-18926
β-strand20116
β-strand23717
β-strand24717
β-strand29918
β-strand30418
β-strand329-33249
β-strand336-33949
β-strand347110
β-strand348-34929
β-strand400110
β-strand420-42121
β-strand428-43031
α-helix445-4506

Molecules and chains

MoleculeChainsTypeLengthOrganismUniProt
WD repeat-containing protein 41Cprotein459Homo sapiensQ9HAD4 (AlphaFold model)
Guanine nucleotide exchange C9orf72Aprotein481Homo sapiensQ96LT7 (AlphaFold model)
Guanine nucleotide exchange protein SMCR8Bprotein937Homo sapiensQ8TEV9 (AlphaFold model)
Sequence of entity 1 (C), FASTA
>6V4U_1 WD repeat-containing protein 41 (chains C)
MLRWLIGGGREPQGLAEKSPLQTIGEEQTQNPYTELLVLKAHHDIVRFLVQLDDYRFASA
GDDGIVVVWNAQTGEKLLELNGHTQKITAIITFPSLESCEEKNQLILTASADRTVIVWDG
DTTRQVQRISCFQSTVKCLTVLQRLDVWLSGGNDLCVWNRKLDLLCKTSHLSDTGISALV
EIPKNCVVAAVGKELIIFRLVAPTEGSLEWDILEVKRLLDHQDNILSLINVNDLSFVTGS
HVGELIIWDALDWTMQAYERNFWDPSPQLDTQQEIKLCQKSNDISIHHFTCDEENVFAAV
GRGLYVYSLQMKRVIACQKTAHDSNVLHVARLPNRQLISCSEDGSVRIWELREKQQLAAE
PVPTGFFNMWGFGRVSKQASQPVKKQQENATSCSLELIGDLIGHSSSVEMFLYFEDHGLV
TCSADHLIILWKNGERESGLRSLRLFQKLEENGDLYLAV
Sequence of entity 2 (A), FASTA
>6V4U_2 Guanine nucleotide exchange C9orf72 (chains A)
MSTLCPPPSPAVAKTEIALSGKSPLLAATFAYWDNILGPRVRHIWAPKTEQVLLSDGEIT
FLANHTLNGEILRNAESGAIDVKFFVLSEKGVIIVSLIFDGNWNGDRSTYGLSIILPQTE
LSFYLPLHRVCVDRLTHIIRKGRIWMHKERQENVQKIILEGTERMEDQGQSIIPMLTGEV
IPVMELLSSMKSHSVPEEIDIADTVLNDDDIGDSCHEGFLLNAISSHLQTCGCSVVVGSS
AEKVNKIVRTLCLFLTPAERKCSRLCEAESSFKYESGLFVQGLLKDSTGSFVLPFRQVMY
APYPTTHIDVDVNTVKQMPPCHEHIYNQRRYMRSELTAFWRATSEEDMAQDTIIYTDESF
TPDLNIFQDVLHRDTLVKAFLDQVFQLKPGLSLRSTFLAQFLLVLHRKALTLIKYIEDDT
QKGKKPFKSLRNLKIDLDLTAEGDLNIIMALAEKIKPGLHSFIFGRPFYTSVQERDVLMT
F
Sequence of entity 3 (B), FASTA
>6V4U_3 Guanine nucleotide exchange protein SMCR8 (chains B)
MISAPDVVAFTKEEEYEEEPYNEPALPEEYSVPLFPFASQGANPWSKLSGAKFSRDFILI
SEFSEQVGPQPLLTIPNDTKVFGTFDLNYFSLRIMSVDYQASFVGHPPGSAYPKLNFVED
SKVVLGDSKEGAFAYVHHLTLYDLEARGFVRPFCMAYISADQHKIMQQFQELSAEFSRAS
ECLKTGNRKAFAGELEKKLKDLDYTRTVLHTETEIQKKANDKGFYSSQAIEKANELASVE
KSIIEHQDLLKQIRSYPHRKLKGHDLCPGEMEHIQDQASQASTTSNPDESADTDLYTCRP
AYTPKLIKAKSTKCFDKKLKTLEELCDTEYFTQTLAQLSHIEHMFRGDLCYLLTSQIDRA
LLKQQHITNFLFEDFVEVDDRMVEKQESIPSKPSQDRPPSSSLEECPIPKVLISVGSYKS
SVESVLIKMEQELGDEEYKEVEVTELSSFDPQENLDYLDMDMKGSISSGESIEVLGTEKS
TSVLSKSDSQASLTVPLSPQVVRSKAVSHRTISEDSIEVLSTCPSEALIPDDFKASYPSA
INEEESYPDGNEGAIRFQASISPPELGETEEGSIENTPSQIDSSCCIGKESDGQLVLPST
PAHTHSDEDGVVSSPPQRHRQKDQGFRVDFSVENANPSSRDNSCEGFPAYELDPSHLLAS
RDISKTSLDNYSDTTSYVSSVASTSSDRIPSAYPAGLSSDRHKKRAGQNALKFIRQYPFA
HPAIYSLLSGRTLVVLGEDEAIVRKLVTALAIFVPSYGCYAKPVKHWASSPLHIMDFQKW
KLIGLQRVASPAGAGTLHALSRYSRYTSILDLDNKTLRCPLYRGTLVPRLADHRTQIKRG
STYYLHVQSMLTQLCSKAFLYTFCHHLHLPTHDKETEELVASRQMSFLKLTLGLVNEDVR
VVQYLAELLKLHYMQESPGTSHPMLRFDYVPSFLYKI

Primary citation

Structure of the C9orf72 ARF GAP complex that is haploinsufficient in ALS and FTD. Su, M.Y., Fromm, S.A., Zoncu, R. et al. Nature (2020) 585:251-255. DOI 10.1038/s41586-020-2633-x · PubMed

Other PDB entries of the same protein (UniProt Q9HAD4 (AlphaFold model), which also has an AlphaFold model), best resolution first:

Browse structure collections

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