Mouse Anti-SMN1 Monoclonal Antibody (CBP2651)

Product Overview

Description

Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of anterior horn cells in the spinal cord and concomitant symmetrical muscle weakness and atrophy (PMID: 16364894). SMA is caused by deletion or mutations of the survival motor neuron (SMN1) gene. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional (PMID: 17355180). A large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein (PMID: 10369862). The level of SMN protein correlates with phenotypic severity of SMA.

Expression Host

Mammalian cells

Immunogen

SMN-Exon7 fusion protein Ag16615

Species Reactivity

Human; Mouse

Clonality

Monoclonal

Host Species

Mouse

Isotype

IgG1

Clone Number

CBP2651

Applications

IF; WB; ELISA

Application Notes

IF : 1:20-1:200; WB : 1:500-1:1000

Research Areas

Neural Signal Transduction

Conjugation

Unconjugated

Product Properties

Form

Liquid

Formulation

PBS, 0.1% sodium azide and 50% glycerol pH 7.3.

Preservatives

Yes

Concentration

1000 μg/ml

Purification

Antigen affinity purification

Purity

>95%, as determined by SDS-PAGE

Shipping

The product is shipped at 4°C. Upon receipt, store it immediately at the temperature recommended below.

Storage

Store at -20°C. Stable for one year after shipment.

Research Use Only

For research use only, not for diagnostic or therapeutic use.

Target

SMN

Official Name

SMN1

Full Name

Survival of motor neuron 1, telomeric

Alternative Names

Component of gems 1; Gemin 1; SMN; SMN1; SMN2; SMNC; SMN-Exon7; SMNT; Survival motor neuron protein; T BCD541

Gene ID

6607(Human); 20595(Mouse); 64301(Rat)

Uniprot ID

Q16637(Human); P97801(Mouse); O35876(Rat)

References

1. Jin, M., Wu, Y., Wang, Y., Yu, D., Yang, M., Yang, F.,... & Chen, T. (2016). MicroRNA-29a promotes smooth muscle cell differentiation from stem cells by targeting YY1. Stem cell research, 17(2), 277-284.

Publications