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SMN Polyclonal antibody, PBS Only

SMN Polyclonal Antibody for WB, IHC, IF/ICC, IP, ELISA

Cat No. 11708-1-PBS

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

WB, IHC, IF/ICC, IP, ELISA

C BCD541, Component of gems 1, Gemin 1, Gemin-1, SMN1

Formulation:  PBS Only
Conjugate:  Unconjugated
Size/Concentration: 
SKU: 

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Freight/Packing: -

Quantity

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Product Information

11708-1-PBS targets SMN in WB, IHC, IF/ICC, IP, ELISA applications and shows reactivity with human, mouse, rat samples.

Tested Reactivity human, mouse, rat
Host / Isotype Rabbit / IgG
Class Polyclonal
Type Antibody
Immunogen

CatNo: Ag2260

Product name: Recombinant human SMN2 protein

Source: e coli.-derived, PGEX-4T

Tag: GST

Domain: 1-282 aa of BC000908

Sequence: MAMSSGGSGGGVPEQEDSVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHALKNGDICETSGKPKTTPKRKPAKKNKSQKKNTAASLQQWKVGDKCSAIWSEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANNIEQNAQENENESQVSTDESENSRSPGNKSDNIKPKSAPWNSFLPPPPPMPGPRLGPGKPGLKFNGPPPPPPPPPPHLLSCWLPPFPSGPPIIPPPPPICPDSLDDADALGSMLISWYMSGYHTGYYMEMLA

Predict reactive species
Full Name survival of motor neuron 2, centromeric
Calculated Molecular Weight 282 aa, 30 kDa
Observed Molecular Weight 38 kDa
GenBank Accession NumberBC000908
Gene Symbol SMN
Gene ID (NCBI) 6607
RRIDAB_2255114
Conjugate Unconjugated
FormLiquid
Purification MethodAntigen affinity purification
UNIPROT IDQ16637
Storage Buffer PBS only, pH 7.3.
Storage ConditionsStore at -80°C.

Background Information

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. This antibody, 11708-1-AP, raised against the recombinant full-length human SMN2 protein, recognizes all isoforms of SMN protein.

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