Modomics - A Database of RNA Modifications

ID Card:

Full name: EKC/KEOPS complex subunit LAGE3
Synonym: ITBA2 CVG5 DXS9951E DXS9879E ESO3 Pcc1
GI: 54041570
UniProt: Q14657
Structures: | 6GWJ |
Alpha Fold Predicted Structure: AF-Q14657-F1


PDB Structures:


6GWJ

Structure Description:

Title: Human OSGEP / LAGE3 / GON7 complex
Classification: RNA BINDING PROTEIN
Technique: X-Ray Diffraction
Resolution: 1.95
R value free: 0.217
R value observed: 0.188
R value work: 0.186

Abstract of the PDB Structure's related Publication:

N 6 -threonyl-carbamoylation of adenosine 37 of ANN-type tRNAs (t 6 A) is a universal modification essential for translational accuracy and efficiency. The t 6 A pathway uses two sequentially acting enzymes, YRDC and OSGEP, the latter being a subunit of the multiprotein KEOPS complex. We recently identified mutations in genes encoding four out of the five KEOPS subunits in children with Galloway-Mowat syndrome (GAMOS), a clinically heterogeneous autosomal recessive disease characterized by early-onset steroid-resistant nephrotic syndrome and microcephaly. Here we show that mutations in YRDC cause an extremely severe form of GAMOS whereas mutations in GON7, encoding the fifth KEOPS subunit, lead to a milder form of the disease. The crystal structure of the GON7/LAGE3/OSGEP subcomplex shows that the intrinsically disordered GON7 protein becomes partially structured upon binding to LAGE3. The structure and cellular characterization of GON7 suggest its involvement in the cellular stability and quaternary arrangement of the KEOPS complex.

Download RCSB-PDB Structures:

Pdb Files   6GWJ.pdb  
Pdbx/mmCIF Files   6GWJ.cif  


Protein sequence:

MRDADADAGGGADGGDGRGGHSCRGGVDTAAAPAGGAPPAHAPGPGRDAASAARGSRMRPHIFTLSVPFPTPLEAEIAHGSLAPDAEPHQRVVGKDLTVSGRILVVRWKAEDCRLLRISVINFLDQLSLVVRTMQRFGPPVSR

Comments:





Alpha Fold Predicted Structure:






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Protein sequence:

M R D A D A D A G G G A D G G D G R G G H S C R G G V D T A A A P A G G A P P A H A P G P G R D A A S A A R G S R M R P H I F T L S V P F P T P L E A E I A H G S L A P D A E P H Q R V V G K D L T V S G R I L V V R W K A E D C R L L R I S V I N F L D Q L S L V V R T M Q R F G P P V S R
20406080100120140MRDADADAGGGADGGDGRGGHSCRGGVDTAAAPAGGAPPAHAPGPGRDAASAARGSRMRPHIFTLSVPFPTPLEAEIAHGSLAPDAEPHQRVVGKDLTVSGRILVVRWKAEDCRLLRISVINFLDQLSLVVRTMQRFGPPVSRSequenceHTSN

Enter the variants

Position

Original

Variant

Secondary Structure Alphabet

  • G: 3-turn helix (310helix)
  • H: α-helix
  • I: 𝝅-helix (5 - turn helix)
  • T: Hydrogen Bonded Turn
  • B: β-sheet
  • S: Bend
  • C: Coil (residues not present in any of the above conformations)
  • N: Not assigned

Download PDB Structures & DSSP Secondary Structures:

Alpha Fold Pdb Files   AF-Q14657-F1.pdb  
Alpha Fold Pdbx/mmCIF Files   AF-Q14657-F1.cif  
DSSP Secondary Structures   Q14657.dssp  





Publications: