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Exalpha
SURF1 Polyclonal Antibody
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Product Details
X1562P
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Full length recombinant human Ssu72 protein
Conjugate
Form
Liquid
Concentration
0.5-2 mg/mL
Purification
Ammonium sulfate precipitation
Storage buffer
PBS
Contains
0.08% sodium azide
Storage conditions
-20°C, Avoid Freeze/Thaw Cycles
Shipping conditions
Ambient (domestic); Wet ice (international)
Product Specific Information
Positive control: MCF-7 and Cos-7 cells.
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: Surfeit locus protein 1
Gene Aliases: CMT4K; SURF-1; SURF1
UniProt ID: (Human) Q15526
Entrez Gene ID: (Human) 6834
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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