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@ARTICLE{Singh:145024,
author = {Singh, Sneha and Nazabal, Alexis and Kaniyappan,
Senthilvelrajan and Pellequer, Jean-Luc and Wolberg, Alisa S
and Imhof, Diana and Oldenburg, Johannes and Biswas, Arijit},
title = {{T}he {P}lasma {F}actor {XIII} {H}eterotetrameric {C}omplex
{S}tructure: {U}nexpected {U}nequal {P}airing within a
{S}ymmetric {C}omplex.},
journal = {Biomolecules},
volume = {9},
number = {12},
issn = {2218-273X},
address = {Basel},
publisher = {MDPI},
reportid = {DZNE-2020-00384},
pages = {765},
year = {2019},
abstract = {Factor XIII (FXIII) is a predominant determinant of clot
stability, strength, and composition. Plasma FXIII
circulates as a pro-transglutaminase with two catalytic A
subunits and two carrier-protective B subunits in a
heterotetramer (FXIII-A2B2). FXIII-A2 and -B2 subunits are
synthesized separately and then assembled in plasma.
Following proteolytic activation by thrombin and
calcium-mediated dissociation of the B subunits, activated
FXIII (FXIIIa) covalently cross links fibrin, promoting clot
stability. The zymogen and active states of the FXIII-A
subunits have been structurally characterized; however, the
structure of FXIII-B subunits and the FXIII-A2B2 complex
have remained elusive. Using integrative hybrid approaches
including atomic force microscopy, cross-linking mass
spectrometry, and computational approaches, we have
constructed the first all-atom model of the FXIII-A2B2
complex. We also used molecular dynamics simulations in
combination with isothermal titration calorimetry to
characterize FXIII-A2B2 assembly, activation, and
dissociation. Our data reveal unequal pairing of individual
subunit monomers in an otherwise symmetric complex, and
suggest this unusual structure is critical for both assembly
and activation of this complex. Our findings enhance
understanding of mechanisms associating FXIII-A2B2 mutations
with disease and have important implications for the
rational design of molecules to alter FXIII assembly or
activity to reduce bleeding and thrombotic complications.},
keywords = {Calcium: pharmacology / Factor XIII: chemistry / HEK293
Cells / Humans / Molecular Docking Simulation / Protein
Conformation / Protein Multimerization / Protein Subunits:
chemistry / Thermodynamics / Thrombin: pharmacology},
cin = {AG Mandelkow 2},
ddc = {570},
cid = {I:(DE-2719)1013015},
pnm = {342 - Disease Mechanisms and Model Systems (POF3-342)},
pid = {G:(DE-HGF)POF3-342},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:31766577},
pmc = {pmc:PMC6995596},
doi = {10.3390/biom9120765},
url = {https://pub.dzne.de/record/145024},
}