ACCÈS RAPIDE AUX LIENS
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.
Informations pour la commande
Nom du produit | Ref. Catalogue | COND. | Prix HT | QTÉ | Favoris | |
Anticorps SURF-1 (B-7) | sc-365060 | 200 µg/ml | $316.00 | |||
SURF-1 (B-7): m-IgGκ BP-HRP Kit | sc-522128 | 200 µg Ab, 40 µg BP | $354.00 | |||
SURF-1 (B-7) peptide neutralisant | sc-365060 P | 100 µg/0.5 ml | $68.00 |