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- SASDFK8: GON7, the fifth subunit of human KEOPS (EKC/KEOPS complex subunit... -

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Basic information

Entry
Database: SASBDB / ID: SASDFK8
SampleGON7, the fifth subunit of human KEOPS
  • EKC/KEOPS complex subunit GON7 (protein), GON7, Homo sapiens
Function / homologyEKC/KEOPS complex subunit GON7, metazoa / Domain of unknown function (DUF4611) / EKC/KEOPS complex / tRNA threonylcarbamoyladenosine modification / nucleolus / nucleoplasm / nucleus / cytosol / EKC/KEOPS complex subunit GON7
Function and homology information
Biological speciesHomo sapiens (human)
CitationDate: 2019 Dec
Title: Defects in t6A tRNA modification due to GON7 and YRDC mutations lead to Galloway-Mowat syndrome
Authors: Arrondel C / Missoury S / Snoek R / Patat J / Menara G / Collinet B / Liger D / Durand D / Gribouval O / Boyer O / Buscara L / Martin G / Machuca E / Nevo F / Lescop E / Braun D / Boschat A ...Authors: Arrondel C / Missoury S / Snoek R / Patat J / Menara G / Collinet B / Liger D / Durand D / Gribouval O / Boyer O / Buscara L / Martin G / Machuca E / Nevo F / Lescop E / Braun D / Boschat A / Sanquer S / Guerrera I / Revy P / Parisot M / Masson C / Boddaert N / Charbit M / Decramer S / Novo R / Macher M / Ranchin B / Bacchetta J / Laurent A / Collardeau-Frachon S / van Eerde A / Hildebrandt F / Magen D / Antignac C / van Tilbeurgh H
Contact author
  • sophia missoury (I2BC-CNRS, Paris, France)

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Structure visualization

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Models

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Sample

SampleName: GON7, the fifth subunit of human KEOPS / Specimen concentration: 6.7 mg/ml
BufferName: 20 mM MES, 200 mM NaCl, 5 mM β-mercaptoethanol / pH: 6.5
Entity #1756Name: GON7 / Type: protein / Description: EKC/KEOPS complex subunit GON7 / Formula weight: 12.59 / Num. of mol.: 1 / Source: Homo sapiens / References: UniProt: Q9BXV9
Sequence:
MGHHHHHHEN LYFQGELLGE YVGQEGKPQK LRVSCEAPGD GDPFQGLLSG VAQMKDMVTE LFDPLVQGEV QHRVAAAPDE DLDGDDEDDA EDENNIDNRT NFDGPSAKRP KTPS

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Experimental information

BeamInstrument name: SOLEIL SWING / City: Saint-Aubin / 国: France / Type of source: X-ray synchrotron / Wavelength: 0.1 Å / Dist. spec. to detc.: 1.8 mm
DetectorName: AVIEX PCCD170170 / Type: CCD
Scan
Title: GON7, the fifth subunit of human KEOPS / Measurement date: Mar 26, 2017 / Cell temperature: 15 °C / Exposure time: 1 sec. / Number of frames: 255 / Unit: 1/A /
MinMax
Q0.0096 0.5003
Distance distribution function P(R)
Sofotware P(R): GNOM 5.0 / Number of points: 517 /
MinMax
Q0.00958007 0.300089
P(R) point1 517
R0 125
Result
Type of curve: sec
Comments: The scattered intensities were displayed on an absolute scale (cm-1) using the scattering of water. SAXS data were normalized to the intensity of the incident beam, averaged and background ...Comments: The scattered intensities were displayed on an absolute scale (cm-1) using the scattering of water. SAXS data were normalized to the intensity of the incident beam, averaged and background subtracted using the program US-SOMO. Frames were examined individually and 30 identical frames were averaged and further processed. The corresponding SEC-elution concentration was approximately 1.5 mg/mL.
ExperimentalStandardPorod
MW14.7 kDa14.7 kDa29 kDa
Volume--46 nm3

P(R)GuinierGuinier error
Forward scattering, I00.01988 0.01971 4.0E-5
Radius of gyration, Rg3.25 nm3.08 nm0.01

MinMax
D-12.5
Guinier point5 47

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