NDLI: The Crystal Structures of Dystrophin and Utrophin Spectrin Repeats: Implications for Domain Boundaries

Content Provider	PubMed Central
Author	Muthu, Muralidharan Richardson, Kylie A. Sutherland-smith, Andrew J.
Editor	Ervasti, James M.
Copyright Year	2012
Abstract	Dystrophin and utrophin link the F-actin cytoskeleton to the cell membrane via an associated glycoprotein complex. This functionality results from their domain organization having an N-terminal actin-binding domain followed by multiple spectrin-repeat domains and then C-terminal protein-binding motifs. Therapeutic strategies to replace defective dystrophin with utrophin in patients with Duchenne muscular dystrophy require full-characterization of both these proteins to assess their degree of structural and functional equivalence. Here the high resolution structures of the first spectrin repeats (N-terminal repeat 1) from both dystrophin and utrophin have been determined by x-ray crystallography. The repeat structures both display a three-helix bundle fold very similar to one another and to homologous domains from spectrin, α-actinin and plectin. The utrophin and dystrophin repeat structures reveal the relationship between the structural domain and the canonical spectrin repeat domain sequence motif, showing the compact structural domain of spectrin repeat one to be extended at the C-terminus relative to its previously defined sequence repeat. These structures explain previous in vitro biochemical studies in which extending dystrophin spectrin repeat domain length leads to increased protein stability. Furthermore we show that the first dystrophin and utrophin spectrin repeats have no affinity for F-actin in the absence of other domains.
Related Links	http://dx.doi.org/10.1371/journal.pone.0040066
Starting Page	40066
File Format	PDF
ISSN	19326203
e-ISSN	19326203
Journal	PLoS ONE
Issue Number	7
Volume Number	7
Language	English
Publisher	Public Library of Science
Publisher Date	2012-07-20
Access Restriction	Open
Rights Holder	Public Library of Science
Subject Keyword	Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Multidisciplinary

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The Crystal Structures of Dystrophin and Utrophin Spectrin Repeats: Implications for Domain Boundaries (2012)

The crystal structures of dystrophin and utrophin spectrin repeats: implications for domain boundaries.

Comparative analysis of the human dystrophin and utrophin gene structures.

Microtubule binding distinguishes dystrophin from utrophin

Dystrophin and utrophin have distinct effects on the structural dynamics of actin

Distinct mechanical properties in homologous spectrin-like repeats of utrophin

G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain.

Alterations of dystrophin associated glycoproteins in the heart lacking dystrophin or dystrophin and utrophin

Spectrin-like repeats from dystrophin and alpha-actinin-2 are not functionally interchangeable (2002).

The Crystal Structures of Dystrophin and Utrophin Spectrin Repeats: Implications for Domain Boundaries

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The Crystal Structures of Dystrophin and Utrophin Spectrin Repeats: Implications for Domain Boundaries (2012)

The crystal structures of dystrophin and utrophin spectrin repeats: implications for domain boundaries.

Comparative analysis of the human dystrophin and utrophin gene structures.

Microtubule binding distinguishes dystrophin from utrophin

Dystrophin and utrophin have distinct effects on the structural dynamics of actin

Distinct mechanical properties in homologous spectrin-like repeats of utrophin

G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain.

Alterations of dystrophin associated glycoproteins in the heart lacking dystrophin or dystrophin and utrophin

Spectrin-like repeats from dystrophin and alpha-actinin-2 are not functionally interchangeable (2002).

The Crystal Structures of Dystrophin and Utrophin Spectrin Repeats: Implications for Domain Boundaries