TIM14_HUMAN » Mitochondrial import inner membrane translocase subunit TIM14

TIM14_HUMAN » Mitochondrial import inner membrane translocase subunit TIM14
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Topology in Mitochondrial inner membrane
Topologyintermembrane space
mitochondrial matrix side
TIM14_HUMAN » Mitochondrial import inner membrane translocase subunit TIM14 » DnaJ homolog subfamily C member 19;
Hydrophobic Thickness 27.8 ± 3.3 Å
Tilt Angle 24 ± 5°
ΔGtransfer -13.0 kcal/mol
ΔGfold -9.9 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 2-23 (2-27)
Pathways none
PDB none
OPM none
Complexes none
Interactions

A4, Complex: A4:TIM14, PubMed

Domains

AA: 65-116, PDBID: 2YS8, Subunit A, Seq Identity:34%, DnaJ domain

UniProt annotation for TIM14_HUMAN » Mitochondrial import inner membrane translocase subunit TIM14
FUNCTION: Probable component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity (By similarity).

SUBUNIT: Probable component of the PAM complex at least composed of a mitochondrial HSP70 protein, GRPEL1 or GRPEL2, TIMM44, TIMM16/PAM16 and TIMM14/DNAJC19.

TISSUE SPECIFICITY: Ubiquitously expressed.

DISEASE: 3-methylglutaconic aciduria 5 (MGA5) OMIM: An autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure and significant increases in urine organic acids, particularly 3- methylglutaconic acid and 3-methylglutaric acid. mutations affecting the gene represented in this entry.

UniProt features for TIM14_HUMAN » Mitochondrial import inner membrane translocase subunit TIM14
CHAIN 2 116 Mitochondrial import inner membrane translocase subunit TIM14.
DOMAIN 62 116 J.
Amino Acid Sequence for TIM14_HUMAN » Mitochondrial import inner membrane translocase subunit TIM14
MASTVVAVGL TIAAAGFAGR YVLQAMKHME PQVKQVFQSL PKSAFSGGYY RGGFEPKMTK REAALILGVS PTANKGKIRD AHRRIMLLNH PDKGGSPYIA AKINEAKDLL EGQAKK