SIP1 (AA 36-156) antibody
Alternatives Western Blotting (WB), Immunofluorescence (IF)
|2 references available|
|Quantity||50 µg (250 µg/ml)|
|Price||Product not available in this region.|
Spinal muscular atrophy (SMA), a commonly fatal autosomal recessive disease, is caused by the degeneration of spinal anterior horn cells. It leads to symmetrical limb and trunk paralysis and muscular atrophy. SMA has been linked to the protein product of the Survival of Motor Neurons (SMN) gene. In greater than 98% of all SMA patients, SMN has been reported to be deleted or mutated. Although its function is unknown, the SMN protein is highly concentrated in novel nuclear structures, termed gems. SIP1 (SMN-interacting protein 1) forms a stable heteromeric complex with SMN and colocalizes with SMN in gems and in the cytoplasm. In SMA, the expression of both proteins is dramatically reduced in motor neurons. Additionally, SMN and SIP1 have been isolated from a 300 kDa protein complex that also contains spliceosomal snRNP proteins. In particular, the SMN-SIP1 complex associates with the spliceosomal snRNAs U1 and U5. Antibodies against the SMN-SIP1 complex interfere with the assembly and nuclear importation of spliceosomal complexes. Thus, it is thought that the SMN-SIP1 complex mediates the formation of spliceosomal snRNPs. However, the exact role of SIP1 in rRNA processing has yet to be determined. This antibody is routinely tested by western blot analysis.
Synonyms: SMN-Interacting Protein 1
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||32 kDa|
Related Products: ABIN968586, ABIN967389
|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|
Liu, Fischer, Wang et al.: "The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins." in: Cell, Vol. 90, Issue 6, pp. 1013-21, 1997 (PubMed).
Fischer, Liu, Dreyfuss: "The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis." in: Cell, Vol. 90, Issue 6, pp. 1023-9, 1997 (PubMed).
|Hosts||Rabbit (7), Mouse (2)|
|Reactivities||Human (7), Mouse (Murine) (3), Rat (Rattus) (2)|
|Applications||Western Blotting (WB) (9), ELISA (6), Immunofluorescence (IF) (3), Immunoprecipitation (IP) (3), Immunocytochemistry (ICC) (2), Immunohistochemistry (IHC) (2), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)) (1)|
|Epitopes||C-Term (2), Internal Region (1), N-Term (1)|