Invitrogen™ SURF1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA530187

Catalog No. PIPA530187

Description

Recommended positive controls: Jurkat. Predicted reactivity: Mouse (84%), Rat (82%). Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

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.

Specifications

• Antigen: SURF1
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: SURF1
• Gene Alias: 0610010F23Rik; Ab1-205; CMT4K; Surf1; Surf-1; surfeit 1; surfeit gene 1; surfeit locus protein 1
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 6834
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Western Blot
• Concentration: 0.83 mg/mL
• Formulation: 0.1M tris glycine with 20% glycerol and 0.01% thimerosal; pH 7
• Gene Accession No.: Q15526
• Gene Symbols: SURF1
• Immunogen: Recombinant protein encompassing a sequence within the center region of human SURF1. The exact sequence is proprietary.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG