MABE230
Anti-SMN2 Antibody, clone SMN-KH
clone SMN-KH, from mouse
Synonym(s):
Survival motor neuron protein, Component of gems 1, Gemin-1
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About This Item
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Recommended Products
biological source
mouse
Quality Level
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
SMN-KH, monoclonal
species reactivity
human
technique(s)
immunohistochemistry: suitable
western blot: suitable
isotype
IgG1κ
NCBI accession no.
UniProt accession no.
shipped in
wet ice
target post-translational modification
unmodified
Gene Information
human ... SMN2(6607)
General description
Survival of Motor Neuron (SMN) is expressed from two linked paralogous genes, SMN1 and SMN2. SMNs are primarily localized in the cytoplasm and nuclear gems of all cells, where they are understood to mediate the assembly of spliceosomal small nuclear ribonucleoprotein particles (snRNPs). They also play a role in the metabolism of snoRNPs and are required for the splicing of pre-mRNA in the nucleus. The progressive loss of functional SMNs in the anterior horn of the spinal cord is a critical cause of Spinal Muscular Atrophy (SMA), an autosomal recessive neuromuscular disease. There are three known types of childhood-onset SMA, in addition to a fourth type that is characterized by adult-onset SMA.
Immunogen
MBP-tagged recombinant protein corresponding to human SMN2.
Application
Anti-SMN2 Antibody, clone SMN-KH is a Mouse Monoclonal Antibody for detection of SMN2 also known as Survival motor neuron protein & has been validated in WB & IHC.
Western Blot Analysis: A representative lot from an independent laboratory detected SMN2 in HeLa cell lysate.
Immunohistochemistry Analysis: A respresentative lot from an independent laboratory detected human SMN2 in SMA type III mouse model thoracic spinal cord tissue. (Hua, Y., et al. (2010). Genes Dev. 24(15):1634-1644.)
Immunohistochemistry Analysis: A respresentative lot from an independent laboratory detected human SMN2 in SMA type III mouse model thoracic spinal cord tissue. (Hua, Y., et al. (2010). Genes Dev. 24(15):1634-1644.)
Quality
Evaluated by Western Blot in HeLa cell lysate.
Western Blot Analysis: 0.5 µg/mL of this antibody detected SMN2 in 10 µg of HeLa cell lysate.
Western Blot Analysis: 0.5 µg/mL of this antibody detected SMN2 in 10 µg of HeLa cell lysate.
Target description
~35 kDa observed
Physical form
Format: Purified
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
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Storage Class Code
12 - Non Combustible Liquids
WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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Suzan M Hammond et al.
JCI insight, 7(24) (2022-11-09)
Antisense oligonucleotides (ASOs) have emerged as one of the most innovative new genetic drug modalities. However, their high molecular weight limits their bioavailability for otherwise-treatable neurological disorders. We investigated conjugation of ASOs to an antibody against the murine transferrin receptor
Anton J Blatnik et al.
Human molecular genetics, 29(21), 3477-3492 (2020-10-20)
Spinal muscular atrophy (SMA) is caused by mutation or deletion of survival motor neuron 1 (SMN1) and retention of SMN2 leading to SMN protein deficiency. We developed an immortalized mouse embryonic fibroblast (iMEF) line in which full-length wild-type Smn (flwt-Smn)
Anna J Kordala et al.
EMBO molecular medicine, 15(11), e17683-e17683 (2023-09-19)
Spinal muscular atrophy (SMA) is a leading genetic cause of infant mortality. The advent of approved treatments for this devastating condition has significantly changed SMA patients' life expectancy and quality of life. Nevertheless, these are not without limitations, and research
Matthew D Howell et al.
Molecular therapy : the journal of the American Society of Gene Therapy, 25(6), 1328-1341 (2017-04-17)
Spinal muscular atrophy (SMA), the leading genetic disease of children, is caused by low levels of survival motor neuron (SMN) protein. Here, we employ A15/283, an antisense oligonucleotide targeting a deep intronic sequence/structure, to examine the impact of restoration of
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