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Transcription profiling of skeletal muscle from wild type and Nebulin knock-out mice

ABSTRACT: Knock out of the Nebulin gene. We compared the Quadriceps of two Nebulin -deficient vs. 2 Wildtype mice. The two KO-WT pairs derived from two litters (F2 generation, background: C57/Bl6 and 129/IB10).

ORGANISM(S): Mus musculus

DISEASE(S): progressive skeletal muscle myopathy

SUBMITTER: Mark McNabb

PROVIDER: E-MEXP-733 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.

Witt Christian C CC Burkart Christoph C Labeit Dietmar D McNabb Mark M Wu Yiming Y Granzier Henk H Labeit Siegfried S

The EMBO journal 20060810 16

The precise assembly of the highly organized filament systems found in muscle is critically important for its function. It has been hypothesized that nebulin, a giant filamentous protein extending along the entire length of the thin filament, provides a blueprint for muscle thin filament assembly. To test this hypothesis, we generated a KO mouse model to investigate nebulin functions in vivo. Nebulin KO mice assemble thin filaments of reduced lengths and approximately 15% of their Z-disks are ab ...[more]

PMID: 16902413

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