ArtNr |
BS2068-50 |
Hersteller |
Bioworld Technology, Inc
|
Menge |
50ul |
Kategorie |
|
Typ |
Antibody Polyclonal |
Applikationen |
WB, IHC |
Specific against |
Human, Mouse, Rat |
Host |
Rabbit |
ECLASS 10.1 |
32160702 |
ECLASS 11.0 |
32160702 |
UNSPSC |
12352203 |
Alias |
Surfeit locus protein 1, SURF1, SURF-1 |
Similar products |
SURF-1 |
Lieferbar |
|
Swiss-Prot |
Q15526 |
Application all |
WB: 1:500ca.1:1000 IHC: 1:50ca.1:200 |
Background |
The SURF-1 protein demonstrates a vital role in the assembly of complex IV (CIV or COX) of the mitochondrial respiratory chain. Expressed in the inner mitochondrial membrane, mutations of the SURF-1 gene generally cause cytochrome c oxidase complex IV deficiency. Shortage of complex IV leads to Leigh syndrome, a severe neurological disorder. Leigh syndrome patients are usually subject to rapidly progressive encephalopathy, characterized by necrotic lesions in subcortical brain regions. SURF-1 mutations correlate to high post-implantation embryonic lethality as well as early-onset mortality of post-natal individuals. Considerable deficit in muscle strength and motor performance is also a profound and isolated defect of SURF-1 activity in skeletal muscle and liver. Heart, brain and skeletal muscle morphological abnormalities frequently occur due to SURF-1 mutations. |
Product |
1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2. |
Purification and Purity |
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE). |
Storage and Stability |
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles. |
Specificity |
SURF-1 (F181) pAb detects endogenous levels of SURF-1 protein. |
WB |
Western blot (WB) analysis of SURF-1 (F181) pAb at 1:500 dilution Lane1:HEK293T whole cell lysate Lane2:Raw264.7 whole cell lysate Lane3:PC12 whole cell lysate |
IHC |
Immunohistochemistry (IHC) analyzes of SURF-1 (F181) pAb in paraffin-embedded liver cancer tissue at 1:100. |
BiowMW |
ca. 33 kDa |
Note |
For research use only, not for use in diagnostic procedure. |
Alternative Name |
Surfeit locus protein 1, SURF1, SURF-1 |
Immunogen |
Synthetic peptide, corresponding to amino acids 150-200 of Human SURF-1. |
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Alle Produkte sind nur für Forschungszwecke bestimmt. Nicht für den menschlichen, tierärztlichen oder therapeutischen Gebrauch.