MABN889MI
MilliporeSigma™ Mouse anti-C9ORF72/C9RANT (poly-GA), Clone: 5E9,
Manufacturer: MilliporeSigma™
Select a Size
| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | MABN889MI-Each-of-1 | In Stock | ₹ 43,318.08 |
MABN889MI - Each of 1
In Stock
Quantity
1
Base Price: ₹ 43,318.08
GST (18%): ₹ 7,797.254
Total Price: ₹ 51,115.334
Antigen
C9ORF72/C9RANT (poly-GA)
Classification
Monoclonal
Formulation
Purified mouse monoclonal IgG1κ in buffer containing 0.1M Tris-Glycine (pH 7.4), 150mM NaCl with 0.05% sodium azide.
Gene Symbols
C9orf72
Immunogen
Polyethylene glycol-conjugated linear peptide containing poly-GA sequence.
Quantity
50 μL
Research Discipline
Neuroscience
Gene ID (Entrez)
NP_001242983
Content And Storage
Stable for one year at 2°C to 8°C from date of receipt.
Isotype
IgG1 κ
Applications
ELISA, Immunohistochemistry, Western Blot
Clone
5E9
Gene Accession No.
Q96LT7
Host Species
Mouse
Purification Method
Protein G Purified
Regulatory Status
RUO
Primary or Secondary
Primary
Target Species
Human
Form
Purified
Related Products
Description
- Specifically detects C9ORF72/C9RANT (poly-GA) clone: 5E9 in Human samples, and it is validated for ELISA, Immunohistochemistry, Western Blotting Expansion of a GGGGCC hexanucleotide repeat sequence in the non-coding region of human chromosome 9 open reading frame 72 or C9orf72 (also known as ALSFTD, FTDALS; Gene ID 203228) is the most common genetic abnormality in familial and sporadic frontotemporal dementia (FTD) and motor neuron disease (MND), with amyotrophic lateral sclerosis (ALS) as the most frequent form
- The number of hexanucleotide repeats in the normal population ranges from 2 to 24, whereas up to several thousand repeats (700 - 4,400 repeats) are found in the pathologically expanded allele
- Unconventional repeat-associated non-ATG-initiated translation (RANT) of the GGGGCC repeats in the three alternate reading frames generates three polypeptides, each composed of repeating units of two amino acids (dipeptide repeats, DPRs), glycine-alanine (GA), glycine-proline (GP), and glycine-arginine (GR), respectively
- These DPR proteins form inclusions particularly in the cerebellar cortex, hippocampus and cerebral neocortex
- These inclusions are also immunoreactive for markers of the ubiquitin proteasome system (UPS), including ubiquitin, ubiquilins, and p62, but is distinct from inclusions containing TDP-43 that is also found in FTLD-TDP (frontotemporal lobar degeneration with TDP-43 pathology) and ALS cases
- Antibodies against poly-GA, poly-GP, and poly-GR sequence are useful for characterizing the neuroanatomical distribution and clinico-pathological association of DPR pathology with C9ORF72 mutations.