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Invitrogen
SURF1 Polyclonal Antibody
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Product Details
PA5-30187
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Recombinant protein encompassing a sequence within the center region of human SURF1. The exact sequence is proprietary.
Conjugate
Form
Liquid
Concentration
0.83 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
0.1M tris glycine, pH 7, with 20% glycerol
Contains
0.01% thimerosal
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
AB_2547661
Product Specific Information
Recommended positive controls: Jurkat.
Predicted reactivity: Mouse (84%), Rat (82%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
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.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
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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