SURF1 polyclonal, anti-human, mouse, rat
€388.00
In stock
SKU
BS71913
Background:
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.
Alternative Name:
Surfeit locus protein 1, SURF1, SURF-1
Application Dilution: WB: 1:500 - 1:2000, IHC: 1:50 - 1:200
Specificity: SURF1 polyclonal antibody detects endogenous levels of SURF1 protein.
Immunogen:
Recombinant protein of human SURF1
MW: 33kDa
Swis Prot.: Q15526
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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.
Alternative Name:
Surfeit locus protein 1, SURF1, SURF-1
Application Dilution: WB: 1:500 - 1:2000, IHC: 1:50 - 1:200
Specificity: SURF1 polyclonal antibody detects endogenous levels of SURF1 protein.
Immunogen:
Recombinant protein of human SURF1
MW: 33kDa
Swis Prot.: Q15526
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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