COX14_HUMAN » Cytochrome c oxidase assembly protein COX14

COX14_HUMAN » Cytochrome c oxidase assembly protein COX14
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Topology in Mitochondrial inner membrane
Topologyintermembrane space
mitochondrial matrix side
COX14_HUMAN » Cytochrome c oxidase assembly protein COX14 »
Hydrophobic Thickness 27.0 ± 2.6 Å
Tilt Angle 3 ± 1°
ΔGtransfer -14.3 kcal/mol
ΔGfold -5.4 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology In
TM Segments 12-30 (8-44)
Pathways none
PDB none
OPM none
Complexes none
Interactions

COA3, Complex: COA3:COX14

Domains

AA: 1-56, Cytochrome oxidase c assembly

UniProt annotation for COX14_HUMAN » Cytochrome c oxidase assembly protein COX14
FUNCTION: Plays a role in the assembly or stability of the cytochrome c oxidase complex (COX). Requires for coordination of the early steps of COX assembly with the synthesis of MT-CO1.

SUBUNIT: Component of some MITRAC complex.

DISEASE: Note=Defects in COX14 may be a cause of a mitochondrial disorder presenting with severe congenital lactic acidosis and dysmorphic features associated with a COX assembly defect. Other features include brain hypertrophy, diffuse alteration of the white-matter myelination, and numerous cavities in the parieto- occipital region, brainstem, and cerebellum, as well as hepatomegaly, hypertrophic cardiomyopathy, renal hypoplasia, and adrenal-gland hyperplasia.

UniProt features for COX14_HUMAN » Cytochrome c oxidase assembly protein COX14
CHAIN 1 57 Cytochrome c oxidase assembly protein COX14.
Amino Acid Sequence for COX14_HUMAN » Cytochrome c oxidase assembly protein COX14
MPTGKQLADI GYKTFSTSMM LLTVYGGYLC SVRVYHYFQW RRAQRQAAEE QKTSGIM