SCO1 Antilichaam

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.

6341

O75880

O75880

Human, Mouse, Rat

Unconjugated

Primary Antibodies

Homeostasis

SCO1 Antibody is affinity chromatography purified via peptide column.

Cat. No. 1303 - Human Brain Tissue Lysate

1 mg/mL

SCO1 Antibody is supplied in PBS containing 0.02% sodium azide.

None

Blue Ice

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.

IgG

SCO1

Cat. No. 10-301 - Human Brain Tissue Slide

SCO cytochrome oxidase deficient homolog 1 (yeast)

Homo sapiens

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.

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.

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.

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.

SCO1 Antibody: SCOD1, SCOD1, Protein SCO1 homolog, mitochondrial

ELISA, WB, IHC-P, IF

8134663

Liquid