Dataset Information


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).

INSTRUMENT(S): 418 [Affymetrix]

ORGANISM(S): Mus musculus  

DISEASE(S): Normal,Progressive Skeletal Muscle Myopathy

SUBMITTER: Christian C Witt   Mark McNabb  Christoph Burkart  Henk Granzier  Dittmar Labeit  Siegfried Labeit  Yiming Wu 

PROVIDER: E-MEXP-733 | ArrayExpress | 2006-11-01


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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]

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