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ProteomicsIdentifying secondary processes implicated in nemaline myopathy pathophysiology
Nemaline myopathy (NM) is a genetically and clinically heterogeneous disease that is diagnosed based on the presence of nemaline rods on skeletal muscle biopsy. While NM has typically been classified by causative genes, disease severity or prognosis cannot be predicted well. The common pathological ...
ORGANISM(S): Mus musculus (Mouse) 
2025-05-06 | PXD042201 | Pride
Nemaline myopathy Neb cko Acta1 
TranscriptomicsThe effect of Nebulin-Deficiency on Skeletal Muscle
Nebulin is a giant filamentous protein that is coextensive with the actin filaments of the skeletal muscle sarcomere. Nebulin mutations are the main cause of nemaline myopathy (NEM), with typical NEM adult patients having low expression of nebulin, yet the roles of nebulin in adult muscle remain po...
ORGANISM(S): Mus musculus 
2015-06-25 | E-GEOD-70213 | biostudies-arrayexpress
TranscriptomicsRNA-seq of splenic B cells from Pbx1-CKO and Ctrl mice
To dissect the molecular regulatory mechanism of Pbx1 in peripheral B cell survival and proliferation, splenic B cells from CKO and Ctrl mice were sorted and stimulated with anti-IgM for 6 hours. Then RNA was extracted for sequencing.
ORGANISM(S): Mus musculus 
2023-03-24 | E-MTAB-12768 | biostudies-arrayexpress
UnknownExpressing a Z-disk nebulin fragment in nebulin-deficient mouse muscle: effects on muscle structure and function.
Not available
S-EPMC6986074 | biostudies-literature
UnknownNebulin stiffens the thin filament and augments cross-bridge interaction in skeletal muscle.
Not available
S-EPMC6187167 | biostudies-literature
UnknownMyostatin Inhibition Using ActRIIB-mFc Does Not Produce Weight Gain or Strength in the Nebulin Conditional KO Mouse.
Not available
S-EPMC6804412 | biostudies-literature
UnknownFunctional Characterization of the Intact Diaphragm in a Nebulin-Based Nemaline Myopathy (NM) Model-Effects of the Fast Skeletal Muscle Troponin Activator tirasemtiv.
Not available
S-EPMC6829460 | biostudies-literature
UnknownNebulin deficiency in adult muscle causes sarcomere defects and muscle-type-dependent changes in trophicity: novel insights in nemaline myopathy.
Not available
S-EPMC4550825 | biostudies-literature
TranscriptomicsThe effect of Nebulin-Deficiency on Skeletal Muscle
Nebulin is a giant filamentous protein that is coextensive with the actin filaments of the skeletal muscle sarcomere. Nebulin mutations are the main cause of nemaline myopathy (NEM), with typical NEM adult patients having low expression of nebulin, yet the roles of nebulin in adult muscle remain poo...
ORGANISM(S): Mus musculus 
2015-06-25 | GSE70213 | GEO
TranscriptomicsRNA-seq of murine bone marrow-derived macrophages (mBMDM) of the wild type (WT) group versus the RBP-J conditionally knockout (CKO) group at 36 hours post HTNV (Hantaan orthohantavirus) infection
Total RNA was extracted from WT and RBP-JCKO mBMDMs at 36 hpi (HTMV, MOI=1), using TRIzol (Invitrogen). Ribosomal RNA was removed using the Ribo-Zero™ kit (Epicentre Biotechnologies). Fragmented RNA (the average length was approximately 200 bp) was subjected to first-strand and second-strand cDNA sy...
ORGANISM(S): Mus musculus 
2022-07-22 | E-MTAB-11926 | biostudies-arrayexpress
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