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SURF1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Invitrogen™

Rabbit Polyclonal Antibody

Manufacturer:  Invitrogen PA549828

Catalog No. PA549828


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Description

Description

The antibody detects endogenous levels of total SURF-1 protein.

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

Specifications

SURF1
Polyclonal
Unconjugated
SURF1
Liquid
SURF1
Synthesized peptide derived from internal of human SURF1.
Antigen affinity chromatography
RUO
Antibody
Polyclonal
Human, Mouse, Rat
Western Blot
1 mg/mL
PBS with 50% glycerol and 0.02% sodium azide; pH 7.4
Q15526, P09925, Q9QXU2
Surfeit locus protein 1, SURF1, SURF-1,
Rabbit
IgG
100 μL
-20°C
Primary
20930, 64463, 6834
Documents
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