Small Nuclear Ribonucleoprotein N (SNRPN) (AA 14-174) antibody

Antigen: Small Nuclear Ribonucleoprotein N (SNRPN)

Binding Site: AA 14-174

Clonality: Monoclonal (8)

Host: Mouse

Reactivity: Human

Application: Western Blotting (WB), Immunofluorescence (IF), Immunohistochemistry (Formalin-fixed Sections) (IHC (f))

Catalog no.: ABIN968086

Additional Information

Alternative name: SMN

Immunogen: Human SMN

Cross-Reactivity: Mouse (Murine), Rat (Rattus), Dog (Canine)

Format: Liquid

Isotype: IgG1

Clone: 8

Description: SMN (survival motor neuron) was discovered as a candidate gene, located in chromosome 5q13, for the fatal autosomal Spinal muscular atrophy (SMA) disorder. The SMN gene was missing or interrupted in a significant number of patients with SMA. The SMN protein is 294 amino acids and migrates with apparent molecular weight of 40 kDa. In addition to the cytoplasm, other studies localized SMN in dots of 0.1-1.0 µm within the nucleus. These novel nuclear structures were named gems and found associated to coiled bodies. It was also found that SMN interacts with the RGG box of hnRNP U and fibrillarin. Therefore, the biochemical function of SMN may be in the regulation of mRNA metabolism.
Synonyms: Survival Motor Neuron

Characteristics: 1. Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2. Please refer to us for technical protocols.
3. Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
4. Source of all serum proteins is from USDA inspected abattoirs located in the United States.

Molecular Weight: 40 kDa

Comments:

Related Products: ABIN968587, ABIN967389

Application Details

Concentration: 250 µg/ml

Purity: Purified

Purification: Purified from tissue culture supernatant or ascites by affinity chromatography.

Buffer: Aqueous buffered solution containing BSA, glycerol.

Preservative: 0.09% Sodium azide.

Storage: Store undiluted at -20° C.

Restrictions: For Research Use only

Images

Western blot analysis of SMN on a HepG2 cell lysate (Human hepatocellular carcinoma, ATCC HB-8065). Lane 1: 1:5000, lane 2: 1:10,000, lane 3: 1:20,000 dilution of the mouse anti-SMN antibody.

Immunofluorescence staining of HeLa cells (Human cervical epitheloid carcinoma, ATCC CCL-2.2).

Immunohistochemical staining of pyrimidal cells in a rat cortex, formalin-fixed paraffin-embedded tissue section, with citrate pre-treatment (magnification, 20X).

Publications

Product: Lefebvre, Bürglen, Reboullet et al.: "Identification and characterization of a spinal muscular atrophy-determining gene." in: Cell, Vol. 80, Issue 1, pp. 155-65, 1995 (PubMed).

Cifuentes-Diaz, Frugier, Tiziano et al.: "Deletion of murine SMN exon 7 directed to skeletal muscle leads to severe muscular dystrophy." in: The Journal of cell biology, Vol. 152, Issue 5, pp. 1107-14, 2001 (PubMed).

Wang, Reddy, Shen: "Higher order arrangement of the eukaryotic nuclear bodies." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 99, Issue 21, pp. 13583-8, 2002 (PubMed).

Claus, Doring, Gringel et al.: "Differential intranuclear localization of fibroblast growth factor-2 isoforms and specific interaction with the survival of motoneuron protein." in: The Journal of biological chemistry, Vol. 278, Issue 1, pp. 479-85, 2002 (PubMed).

Côté, Boisvert, Boulanger et al.: "Sam68 RNA binding protein is an in vivo substrate for protein arginine N-methyltransferase 1." in: Molecular biology of the cell, Vol. 14, Issue 1, pp. 274-87, 2003 (PubMed).