SCO2_HUMAN » Protein SCO2 homolog, mitochondrial

SCO2_HUMAN » Protein SCO2 homolog, mitochondrial
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Topology in Mitochondrial inner membrane
Topologyintermembrane space
mitochondrial matrix side
SCO2_HUMAN » Protein SCO2 homolog, mitochondrial »
Hydrophobic Thickness 24.4 ± 2.8 Å
Tilt Angle 0 ± 0°
ΔGtransfer -25.6 kcal/mol
ΔGfold -7.7 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology In
TM Segments 59-79 (55-83)
Pathways none
PDB 2rli (100-266)
OPM none
Complexes none
Interactions

A4, Complex: A4:SCO2

SRPRB, Complex: SRPRB:SCO2

STX7, Complex: STX7:SCO2

THY1, Complex: THY1:SCO2

Domains

AA: 64-245, PDBID: 2RLI, Subunit A, Seq Identity:100%, SCO1/SenC

UniProt annotation for SCO2_HUMAN » Protein SCO2 homolog, mitochondrial
FUNCTION: Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).

TISSUE SPECIFICITY: Ubiquitous.

DISEASE: Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 1 (CEMCOX1) OMIM: A disorder characterized by hypotonia, developmental delay, hypertrophic cardiomyopathy, lactic acidosis, gliosis, neuronal loss in basal ganglia, brainstem and spinal cord, and cytochrome c oxidase deficiency. mutations affecting the gene represented in this entry.

DISEASE: Myopia 6 (MYP6) OMIM: A refractive error of the eye, in which parallel rays from a distant object come to focus in front of the retina, vision being better for near objects than for far. mutations affecting the gene represented in this entry.

UniProt features for SCO2_HUMAN » Protein SCO2 homolog, mitochondrial
TRANSIT 1 41 Mitochondrion.
CHAIN 42 266 Protein SCO2 homolog, mitochondrial.
DOMAIN 85 259 Thioredoxin.
Amino Acid Sequence for SCO2_HUMAN » Protein SCO2 homolog, mitochondrial
MLLLTRSPTA WHRLSQLKPR VLPGTLGGQA LHLRSWLLSR QGPAETGGQG QPQGPGLRTR LLITGLFGAG LGGAWLALRA EKERLQQQKR TEALRQAAVG QGDFHLLDHR GRARCKADFR GQWVLMYFGF THCPDICPDE LEKLVQVVRQ LEAEPGLPPV QPVFITVDPE RDDVEAMARY VQDFHPRLLG LTGSTKQVAQ ASHSYRVYYN AGPKDEDQDY IVDHSIAIYL LNPDGLFTDY YGRSRSAEQI SDSVRRHMAA FRSVLS