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Mouse Anti-SPEG Recombinant Antibody (CBXS-4433) (CBMAB-S1675-CQ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXS-4433
Antibody Isotype
IgG1, κ
Application
ELISA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1, κ
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
SPEG Complex Locus
Introduction
This gene encodes a protein with similarity to members of the myosin light chain kinase family. This protein family is required for myocyte cytoskeletal development. Along with the desmin gene, expression of this gene may be controlled by the desmin locus control region. Mutations in this gene are associated with centronuclear myopathy 5.
Entrez Gene ID
10290
UniProt ID
Q15772
Alternative Names
SPEG Complex Locus; Aortic Preferentially Expressed Protein 1; Aortic Preferentially Expressed Gene 1; EC 2.7.11.1; APEG-1; APEG1; Nuclear Protein, Marker For Differentiated Aortic Smooth Muscle And Down-Regulated With Vascular Injury; Striated Muscle Preferentially Expressed Protein Kinase;
Function
Isoform 3 may have a role in regulating the growth and differentiation of arterial smooth muscle cells.
Biological Process
Biological Process muscle cell differentiationManual Assertion Based On ExperimentIBA:GO_Central
Biological Process muscle organ developmentManual Assertion Based On ExperimentTAS:ProtInc
Biological Process negative regulation of cell population proliferationManual Assertion Based On ExperimentTAS:ProtInc
Biological Process protein phosphorylationIEA:InterPro
Cellular Location
Isoform 3
Nucleus
Involvement in disease
Myopathy, centronuclear, 5 (CNM5):
A form of centronuclear myopathy, a congenital muscle disorder characterized by progressive muscular weakness and wasting involving mainly limb girdle, trunk, and neck muscles. It may also affect distal muscles. Weakness may be present during childhood or adolescence or may not become evident until the third decade of life. Ptosis is a frequent clinical feature. The most prominent histopathologic features include high frequency of centrally located nuclei in muscle fibers not secondary to regeneration, radial arrangement of sarcoplasmic strands around the central nuclei, and predominance and hypotrophy of type 1 fibers. CNM5 features include severe neonatal hypotonia with respiratory insufficiency, difficulty feeding, and delayed motor development. Some patients die in infancy, and some develop dilated cardiomyopathy.
PTM
May be autophosphorylated.
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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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