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Invitrogen™ SURF1 Polyclonal Antibody
Rabbit Polyclonal Antibody
Supplier: Invitrogen™ PA5110448
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
SURF1 | |
Polyclonal | |
Unconjugated | |
SURF1 | |
0610010F23Rik; Ab1-205; CMT4K; Surf1; Surf-1; surfeit 1; surfeit gene 1; surfeit locus protein 1 | |
Rabbit | |
Affinity chromatography | |
RUO | |
20930, 64463, 6834 | |
-20°C, Avoid Freeze/Thaw Cycles | |
Liquid |
ELISA, Western Blot | |
2.15 mg/mL | |
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3 | |
P09925, Q15526, Q9QXU2 | |
SURF1 | |
Recombinant fusion protein containing a sequence corresponding to amino acids 80-275 of human SURF1 (NP_003163.1). | |
100 μL | |
Primary | |
Human, Mouse, Rat | |
Antibody | |
IgG |
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