delta Sarcoglycan Recombinant Rabbit Monoclonal Antibody [JM61-10]
Catalog# ET1703-88
delta Sarcoglycan Recombinant Rabbit Monoclonal Antibody [JM61-10]
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WB
-
IP
-
IHC-P
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Human
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unconjugated
Overview
Product Name
delta Sarcoglycan Recombinant Rabbit Monoclonal Antibody [JM61-10]
Antibody Type
Recombinant Rabbit monoclonal Antibody
Immunogen
Synthetic peptide within Human delta Sarcoglycan aa 221-265 / 289.
Species Reactivity
Human
Validated Applications
WB, IP, IHC-P
Molecular Weight
32 kDa
Positive Control
Human lung tissue lysate, human skeletal muscle tissue lysate, human stomach carcinoma tissue.
Conjugation
unconjugated
Clone Number
JM61-10
RRID
Product Features
Form
Liquid
Concentration
Storage Instructions
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). Store at -20℃ long term.
Storage Buffer
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Isotype
IgG
Purification Method
Protein A affinity purified.
Application Dilution
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WB
-
1:500-1:2,000
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IHC-P
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1:50-1:200
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IP
-
Use at an assay dependent concentration.
Target
Function
Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix. Defects in SGCD are the cause of limb-girdle muscular dystrophy type 2F (LGMD2F) [MIM:601287]. LGMD2F is an autosomal recessive disorder. Defects in SGCD are the cause of cardiomyopathy dilated type 1L (CMD1L) [MIM:606685]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Background References
1. Vanhoutte D et al. Thrombospondin expression in myofibers stabilizes muscle membranes. Elife 5:N/A (2016).
Sequence Similarity
Belongs to the sarcoglycan beta/delta/gamma/zeta family.
Tissue Specificity
Most strongly expressed in skeletal and cardiac muscle. Also detected in smooth muscle. Weak expression in brain and lung.
Post-translational Modification
Glycosylated.; Disulfide bonds are present.
Subcellular Location
Cytoskeleton, sarcolemma.
UNIPROT
Synonyms
35 kDa dystrophin associated glycoprotein antibody
35 kDa dystrophin-associated glycoprotein antibody
35DAG antibody
CMD1L antibody
DAGD antibody
Delta-sarcoglycan antibody
Delta-SG antibody
Dystrophin associated glycoprotein delta sarcoglycan antibody
LGMD2F antibody
MGC22567 antibody
ExpandImages
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Western blot analysis of delta Sarcoglycan on different lysates. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (ET1703-88, 1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1:5,000 dilution was used for 1 hour at room temperature.
Positive control:
Lane 1: Human lung tissue lysate
Lane 2: Human skeletal muscle tissue lysate -
Immunohistochemical analysis of paraffin-embedded human stomach carcinoma tissue using anti-delta Sarcoglycan antibody. The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 8.0-8.4) for 20 minutes.The tissues were blocked in 5% BSA for 30 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1703-88, 1/50) for 30 minutes at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"