Invitrogen™ SURF1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA5110449

Product Code. 17178373

Description

Immunogen sequence: QVQRRKWKLN LIAELESRVL AEPVPLPADP MELKNLEYRP VKVRGCFDHS KELYMMPRTM VDPVREAREG GLISSSTQSG AYVVTPFHCT DLGVTILVNR GFVPRKKVNP ETRQKGQIEG EVDLIGMVRL TETRQPFVPE NNPERNHWHY RDLEAMARIT GAEPIFIDAN FQSTVPGGPI GGQTRVTLRN EHLQYI.

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: Affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 20930, 64463, 6834
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Form: Liquid

• Applications: ELISA, Western Blot
• Concentration: 1.21 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: Recombinant fusion protein containing a sequence corresponding to amino acids 80-275 of human SURF1 (NP_003163.1).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG