BS2068

antibody from Bioworld Technology, Inc

Targeting: SURF1

Immunohistochemistry

Antibody data

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Validation data

Product number: BS2068 - Provider product page
Provider: Bioworld Technology, Inc
Product name: SURF 1 (F181) polyclonal antibody
Antibody type: Polyclonal
Antigen: Synthetic peptide, corresponding to amino acids 150-200 of Human SURF-1.
Description: 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.
Reactivity: Human, Mouse, Rat
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.

SURF1 protein structure - BS2068 shown in red.