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TIMM50

Domain

The FCP1 homology domain does not contain the canonical D-x-D-x-[TV] active site, suggesting that it probably does not display phosphatase activity in vivo.

Function

Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. Has some phosphatase activity in vitro; however such activity may not be relevant in vivo.

Isoform 2

May participate in the release of snRNPs and SMN from the Cajal body.

Involvement in disease

3-methylglutaconic aciduria 9

MGCA9

An autosomal recessive disease characterized by early-onset seizures, severely delayed psychomotor development and intellectual disability. Patients have hypotonia or spasticity, and laboratory investigations show increased serum lactate and 3-methylglutaconic aciduria.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence similarities

Belongs to the TIM50 family.

Tissue specificity

Widely expressed. Expressed at higher level in brain, kidney and liver (at protein level).

Cellular localization

  • Mitochondrion inner membrane
  • Single-pass membrane protein
  • Isoform 2
  • Nucleus speckle
  • Nuclear and enriched in speckles with snRNPs.

Alternative names

  • Mitochondrial import inner membrane translocase subunit TIM50
  • TIM50
  • TIMM50
  • PRO1512

Target type

Proteins

Molecular weight

39646Da