DATASHEET
Host:
Rabbit
Target Protein:
NDUFS7
Immunogen Range:
101-160/213
Clonality:
Polyclonal
Isotype:
IgG
Source:
KLH conjugated synthetic peptide derived from human NDUFS7
Purification:
Purified by Protein A.
Storage Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
Background:
Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimately leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
VALIDATION IMAGES
Paraformaldehyde-fixed, paraffin embedded Mouse skeletal muscle; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with NDUFS7 Polyclonal Antibody, Unconjugated (bs-11734R) at 1:400 overnight at 4°C, followed by a conjugated secondary antibody for 20 minutes and DAB staining.
Lane 1: Mouse Heart lysates probed with NDUFS7 Polyclonal Antibody, Unconjugated (bs-11734R) at 1:300 overnight at 4˚C. Followed by a conjugated secondary antibody at 1:10000 for 60 min at 37˚C.