RGD Reference Report - Development of rabbit monoclonal antibodies for detection of alpha-dystroglycan in normal and dystrophic tissue. - Rat Genome Database

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Development of rabbit monoclonal antibodies for detection of alpha-dystroglycan in normal and dystrophic tissue.

Authors: Fortunato, MJ  Ball, CE  Hollinger, K  Patel, NB  Modi, JN  Rajasekaran, V  Nonneman, DJ  Ross, JW  Kennedy, EJ  Selsby, JT  Beedle, AM 
Citation: Fortunato MJ, etal., PLoS One. 2014 May 13;9(5):e97567. doi: 10.1371/journal.pone.0097567. eCollection 2014.
RGD ID: 11537406
Pubmed: PMID:24824861   (View Abstract at PubMed)
PMCID: PMC4019581   (View Article at PubMed Central)
DOI: DOI:10.1371/journal.pone.0097567   (Journal Full-text)

Alpha-dystroglycan requires a rare O-mannose glycan modification to form its binding epitope for extracellular matrix proteins such as laminin. This functional glycan is disrupted in a cohort of muscular dystrophies, the secondary dystroglycanopathies, and is abnormal in some metastatic cancers. The most commonly used reagent for detection of alpha-dystroglycan is mouse monoclonal antibody IIH6, but it requires the functional O-mannose structure for recognition. Therefore, the ability to detect alpha-dystroglycan protein in disease states where it lacks the full O-mannose glycan has been limited. To overcome this hurdle, rabbit monoclonal antibodies against the alpha-dystroglycan C-terminus were generated. The new antibodies, named 5-2, 29-5, and 45-3, detect alpha-dystroglycan from mouse, rat and pig skeletal muscle by Western blot and immunofluorescence. In a mouse model of fukutin-deficient dystroglycanopathy, all antibodies detected low molecular weight alpha-dystroglycan in disease samples demonstrating a loss of functional glycosylation. Alternately, in a porcine model of Becker muscular dystrophy, relative abundance of alpha-dystroglycan was decreased, consistent with a reduction in expression of the dystrophin-glycoprotein complex in affected muscle. Therefore, these new rabbit monoclonal antibodies are suitable reagents for alpha-dystroglycan core protein detection and will enhance dystroglycan-related studies.

RGD Manual Disease Annotations    Click to see Annotation Detail View

Gene Ontology Annotations    Click to see Annotation Detail View

Cellular Component
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
sarcolemma  IDA 11537406 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Dag1  (dystroglycan 1)
Fktn  (fukutin)

Genes (Mus musculus)
Dag1  (dystroglycan 1)
Fktn  (fukutin)

Genes (Homo sapiens)
DAG1  (dystroglycan 1)
FKTN  (fukutin)


Additional Information