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Invitrogen
SURF1 Polyclonal Antibody
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Please note: We are reviewing Western blot images included in the antibody testing data in our catalog, including those provided by third parties. Unless expressly labeled or annotated as “raw-unedited”, Western blot images included in the antibody testing data in our catalog may have been edited, optimized or otherwise adjusted for presentation.
Product Details
PA5-106501
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
A synthesized peptide derived from human SURF1(Accession Q15526), corresponding to amino acid residues Y161-E211.
Conjugate
Unconjugated
Unconjugated
Unconjugated
Form
Liquid
Concentration
1 mg/mL
Amount
100 µg
Purification
Affinity chromatography
Storage buffer
PBS, pH 7.4, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
Product Specific Information
Antibody detects endogenous levels of total SURF1.
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: SURF1; surfeit 1; unnamed protein product
Gene Aliases: 0610010F23Rik; Ab1-205; CMT4K; MC4DN1; SHY1; Surf-1
UniProt ID: (Human) Q15526, (Mouse) P09925, (Rat) Q9QXU2
Entrez Gene ID: (Human) 6834, (Mouse) 20930, (Rat) 64463
Disclaimer
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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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