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Invitrogen™ SURF1 Polyclonal Antibody
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Catalog No. PIPA530187
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100 μL
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PIPA530187 100 μL
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Catalog No. PIPA530187 Supplier Invitrogen™ Supplier No. PA530187
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Description

Rabbit Polyclonal Antibody

Recommended positive controls: Jurkat. Predicted reactivity: Mouse (84%), Rat (82%). Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen SURF1
Applications Western Blot
Classification Polyclonal
Concentration 0.83 mg/mL
Conjugate Unconjugated
Formulation 0.1M tris glycine with 20% glycerol and 0.01% thimerosal; pH 7
Gene SURF1
Gene Accession No. Q15526
Gene Alias 0610010F23Rik; Ab1-205; CMT4K; Surf1; Surf-1; surfeit 1; surfeit gene 1; surfeit locus protein 1
Gene Symbols SURF1
Host Species Rabbit
Immunogen Recombinant protein encompassing a sequence within the center region of human SURF1. The exact sequence is proprietary.
Purification Method Antigen affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 6834
Target Species Human
Content And Storage Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Product Type Antibody
Form Liquid
Isotype IgG
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