SURF1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Supplier:  Proteintech Group Inc 153791AP150UL

Catalog No. 50-173-0189

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.

Specifications

• Antigen: SURF1
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: SURF1
• Gene Alias: SURF 1, SURF1, surfeit 1, Surfeit locus protein 1
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 20930, 64463, 6834
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Immunoprecipitation, Western Blot
• Concentration: 0.17 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene Accession No.: P09925, Q15526, Q9QXU2
• Gene Symbols: Surf1
• Immunogen: SURF1 Fusion Protein Ag3959
• Quantity: 150 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG