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Proteintech
SURF1 Polyclonal Antibody
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Product Details
15379-1-AP
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse
Published species
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
SURF1 Fusion Protein Ag3959 (1-300 aa encoded by BC028314)
Conjugate
Form
Liquid
Concentration
0.17 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice (domestic); Dry ice (international)
Product Specific Information
Immunogen sequence: MAAVAALQL GLRAAGLGRA PASAAWRSVL RVSPRPGVAW RPSRCGSSAA EASATKAEDD SFLQWVLLLI PVTAFGLGTW QVQRRKWKLN LIAELESRVL AEPVPLPADP MELKNLEYRP VKVRGCFDHS KELYMMPRTM VDPVREAREG GLISSSTQSG AYVVTPFHCT DLGVTILVNR GFVPRKKVNP ETRQKGQIEG EVDLIGMVRL TETRQPFVPE NNPERNHWHY RDLEAMARIT GAEPIFIDAN FQSTVPGGPI GGQTRVTLRN EHLQYIVTWY GLSAATSYLW FKKFLRGTPG V (1-300 aa encoded by BC028314)
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.
Bioinformatics
Protein Aliases: Surfeit locus protein 1
Gene Aliases: 0610010F23Rik; CMT4K; SURF-1; SURF1
UniProt ID: (Human) Q15526, (Mouse) P09925
Entrez Gene ID: (Human) 6834, (Mouse) 20930
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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