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PDBsum entry 6h4l
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Structural protein
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PDB id
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6h4l
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References listed in PDB file
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Key reference
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Title
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Structural diversity in the atomic resolution 3d fingerprint of the titin m-Band segment.
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Authors
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S.D.Chatziefthimiou,
P.Hornburg,
F.Sauer,
S.Mueller,
D.Ugurlar,
E.R.Xu,
M.Wilmanns.
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Ref.
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PLoS One, 2019,
14,
e0226693.
[DOI no: ]
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PubMed id
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Abstract
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In striated muscles, molecular filaments are largely composed of long protein
chains with extensive arrays of identically folded domains, referred to as
"beads-on-a-string". It remains a largely unresolved question how
these domains have developed a unique molecular profile such that each carries
out a distinct function without false-positive readout. This study focuses on
the M-band segment of the sarcomeric protein titin, which comprises ten
identically folded immunoglobulin domains. Comparative analysis of
high-resolution structures of six of these domains ‒ M1, M3, M4, M5, M7, and
M10 ‒ reveals considerable structural diversity within three distinct loops
and a non-conserved pattern of exposed cysteines. Our data allow to structurally
interpreting distinct pathological readouts that result from
titinopathy-associated variants. Our findings support general principles that
could be used to identify individual structural/functional profiles of hundreds
of identically folded protein domains within the sarcomere and other densely
crowded cellular environments.
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