Complex IV subunit SURF1 monoclonal antibody
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Catalog No. MS403
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$325.00 - 100 µg
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UniProt Number:
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Q15526
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Alternate Names:
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Surfeit locus protein 1, SURF-1
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Structure and Function:
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SURF1 is an important assembly factor for cytochrome c oxidase, which is thought to participate in the folding of subunit II and parallel Cu2+ insertion into this subunit.
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Disease Associations:
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Mutations of SURF1, particularly ones that result in an absence or near absence of the polypeptide, are the leading cause of cytochrome c oxidase deficiency. Cytochrome c oxidase deficiency is associated with Leigh syndrome (LS), which can be caused by abnormalities in SURF1 and is characterized by necrotic lesions that are located symmetrically in both sides the subcortical brain.
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Product Specifications
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Applications:
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Western blotting
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Species Reactivity:
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human
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Host Species:
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mouse
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Isotype:
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IgG1, κ
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Clone ID:
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21H2BG4
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Concentration:
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1.0 mg/mL in Hepes-Buffered Saline (HBS) with 0.02% azide as a preservative.
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Suggested Working Concentration:
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1 µg/mL for Western blotting
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Storage Conditions:
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Store at 4°C. Do not freeze.
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Country of Origin:
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USA
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WB Images
(click to enlarge)
Figure 1. Complex IV assembly factor Surf1 identification. MS403 mAb can be used to verify the presence of Complex IV assembly factor Surf1. Blotted alongside are mAbs against the ATP synthase subunit F1α (MS502), an abundant mitochondrial protein acting as a control for gel loading. To indicate the assembly state of the cytochrome c oxidase, mAbs against the nuclear cytochrome c oxidase subunit Va (MS409) and the mitochondrially encoded COX subunit II (MS405) are also blotted.
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