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SCO1 Antibody

CAT: 0223-4043-01Size: 0.02 mgDry Ice: NoHazardous: No
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CAT#:0223-4043-01Size:0.02 mg
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Background
SCO1 Antibody: Synthesis of cytochrome c oxidase 1 was initially identified in yeast as one of two cytochrome c oxidase (COX) assembly proteins that enable the assembly of cytochrome c holoenzyme, a complex that catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. Like their yeast homologs, the function of both SCO1 and SCO2 are dependent on copper ion binding. Mutations in either gene can lead to cytochrome c oxidase respiratory chain defects, with a missense mutation in human SCO1 (P174L) associated with a fatal neonatal hepatopathy when the second allele is also non-functional, suggesting the pathology is due to loss of function. It has been suggested that this mutation alters the SCO1 affinity for the copper (I) ion, thus impairing the efficiency of copper transfer to the cytochrome c oxidase. At least two isoforms of SCO1 are known to exist and both are recognized by the SCO1 antibody. This SCO1 antibody has no cross-reactivity to SCO2.
NCBI Gene ID
6341
Swiss Prot
O75880
Accession Number
O75880
Host
Rabbit
Reactivity
Human, Mouse, Rat
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Homeostasis
Purification
SCO1 Antibody is affinity chromatography purified via peptide column.
Positive Control
Cat. No. 1303 - Human Brain Tissue Lysate
Concentration
1 mg/mL
Buffer
SCO1 Antibody is supplied in PBS containing 0.02% sodium azide.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
SCO1 antibody can be stored at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Fragment
IgG
Symbol
SCO1
Positive Control 2
Cat. No. 10-301 - Human Brain Tissue Slide
NCBI Official Name
SCO cytochrome oxidase deficient homolog 1 (yeast)
NCBI Organism
Homo sapiens
Background Reference 01
Glerum DM, Shtanko A, and Tzagoloff A. SCO1 and SCO2 act as high copy suppressors of a mitochondrial copper recruitment defect in Saccharomyces cerevisiae.J. Biol. Chem.1996; 271:20531-5.
Background Reference 02
Horng Y-C, Leary SC, Cobine PA, et al. Human Sco1 and Sco2 function as copper-binding proteins. J. Biol. Chem.2005; 280:34113-22.
Background Reference 03
Valnot I, Osmond S, Gigarel N. Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy. Am. J. Hum. Genet.2000; 67:1104-9.
Background Reference 04
Banci L, Bertini I, Ciofi-Baffoni S, et al. Human Sco1 functional studies and pathological implications of the P174L mutant. Proc. Natl. Acad. Sci. USA2007; 104:15-20.
Other Product Names
SCO1 Antibody: SCOD1, SCOD1, Protein SCO1 homolog, mitochondrial
Tested Applications
ELISA, WB, IHC-P, IF
Protein ID
8134663
Physical Properties
Liquid

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