Alternative Name(s):

Cytochrome oxidase deficient homolog 2; MGC125823; MGC125825; OTTHUMP00000196774; OTTHUMP00000196775; Protein SCO2 homolog; mitochondrial; SCO (cytochrome oxidase deficient; yeast) homolog 2; SCO 1L; SCO 2; SCO cytochrome oxidase deficient homolog 2 (yeast); SCO cytochrome oxidase deficient homolog 2; SCO1L; SCO2; SCO2_HUMAN; Synthesis of cytochrome c oxidase 2

Species: (Organism)

Homo sapiens (Human)

Gene Names:

SCO2

Tag info:

N-terminal 6xHis-SUMO-tagged

Target Protein AA Sequence:

PAETGGQGQPQGPGLRTRLLITGLFGAGLGGAWLALRAEKERLQQQKRTEALRQAAVGQGDFHLLDHRGRARCKADFRGQWVLMYFGFTHCPDICPDELEKLVQVVRQLEAEPGLPPVQPVFITVDPERDDVEAMARYVQDFHPRLLGLTGSTKQVAQASHSYRVYYNAGPKDEDQDYIVDHSIAIYLLNPDGLFTDYYGRSRSAEQISDSVRRHMAAFRSVLS

Expression Region:

43-266aa

Subcellular Location:

Mitochondrion

Tissue Specificity:

Ubiquitous.

Protein Length:

Full Length of Mature Protein

Pathway:

Mol. Weight:

41.1 kDa

Purity:

Greater than 90% as determined by SDS-PAGE.

Form:

Liquid or Lyophilized powder

Buffer:

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Research Areas:

Metabolism

Function:

Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).

Involvement in disease:

Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 1 (CEMCOX1); Myopia 6 (MYP6); Leigh syndrome (LS)

Relevance:

Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).

Reconstitution:

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Protein Families:

SCO1/2 family

Reference:

Mutations in SCO2 are associated with autosomal-dominant high-grade myopia.Tran-Viet K.N., Powell C., Barathi V.A., Klemm T., Maurer-Stroh S., Limviphuvadh V., Soler V., Ho C., Yanovitch T., Schneider G., Li Y.J., Nading E., Metlapally R., Saw S.M., Goh L., Rozen S., Young T.L.Am. J. Hum. Genet. 92:820-826(2013)