Antibody data

Product number
BS8394
Provider
Bioworld Technology, Inc
Product name
SCO1 polyclonal antibody
Provider product page
Bioworld Technology, Inc - BS8394
Antibody type
Polyclonal
Antigen
Recombinant full length Human SCO1.
Description
The SCO1 and SCO2 protein homologs belong to the SCO1/2 family of proteins. SCO1 and SCO2 both localize to the mitochondrion and are inner membrane proteins crucial for copper insertion or transport to the active site of cytochrome c oxidase (COX). COX is a crucial component in energy production because it functions as the terminal enzyme in the respiratory chain. SCO1 is predominantly expressed in highly oxidative phosphorylation tissues such as brain, heart and muscle, while SCO2 is ubiquitously expressed. Defects in the gene encoding for SCO1 may cause cytochrome c oxidase deficiency, a hetero-genous disorder. Defects in the gene encoding for SCO2 may cause cardioencephalomyopathy with cytochrome c oxidase deficiency, a fatal infantile disorder characterized by hypertrophic cardiomyopathy, lactic acidosis and gliosis.
Reactivity
Human
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Provider Type Product Number
- No reagents -