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Mouse Anti-SGCA Recombinant Antibody (CBXS-4805) (CBMAB-S2031-CQ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXS-4805
Antibody Isotype
IgG2b, κ
Application
ELISA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2b, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.2
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
SGCA
Introduction
This gene encodes a component of the dystrophin-glycoprotein complex (DGC), which is critical to the stability of muscle fiber membranes and to the linking of the actin cytoskeleton to the extracellular matrix. Its expression is thought to be restricted to striated muscle. Mutations in this gene result in type 2D autosomal recessive limb-girdle muscular dystrophy. Multiple transcript variants encoding different isoforms have been found for this gene.
Entrez Gene ID
6442
UniProt ID
Q16586
Alternative Names
Sarcoglycan Alpha; Adhalin; Sarcoglycan, Alpha (50kDa Dystrophin-Associated Glycoprotein); 50 KDa Dystrophin-Associated Glycoprotein; Limb Girdle Muscular Dystrophy 2D; Dystroglycan-2; 50kD DAG; Alpha-SG;
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.
Biological Process
Biological Process muscle contractionManual Assertion Based On ExperimentTAS:ProtInc
Biological Process muscle organ developmentManual Assertion Based On ExperimentTAS:ProtInc
Biological Process response to denervation involved in regulation of muscle adaptationIEA:Ensembl
Biological Process skeletal muscle tissue regenerationIEA:Ensembl
Cellular Location
Cell membrane, sarcolemma
Cytoplasm, cytoskeleton
Involvement in disease
Muscular dystrophy, limb-girdle, autosomal recessive 3 (LGMDR3):
An autosomal recessive degenerative myopathy characterized by progressive muscle wasting from early childhood with loss of independent ambulation by teenage years. Muscle biopsy shows necrosis, decreased immunostaining for alpha sarcoglycan, and adhalin deficiency.
Topology
Extracellular: 24-290
Helical: 291-311
Cytoplasmic: 312-387
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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