Synthesized peptide derived from the Internal region of human Cytochrome c1.
Host:
Rabbit
Clonality:
Polyclonal
Conjugate:
Unconjugated
Purification:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:
1mg / ml
Formulation:
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Storage:
Store at -20?C / 1 year
Target
Function:
This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain.
Involvement in Disease:
Mitochondrial complex III deficiency, nuclear 6: An autosomal recessive disorder caused by mitochondrial dysfunction. It is characterized by onset in early childhood of episodic acute lactic acidosis, ketoacidosis, and insulin-responsive hyperglycemia, usually associated with infection. Laboratory studies show decreased activity of mitochondrial complex III. Psychomotor development is normal.