Recombinant Human ATP-dependent zinc metalloprotease YME1L1 (YME1L1), Truncated

Uniprot Id

Q96TA2

Target Species

Human

Target Name

YME1L1

Target Full Name

ATP-dependent zinc metalloprotease YME1L1

Target Function

ATP-dependent metalloprotease that catalyzes the degradation of folded and unfolded proteins with a suitable degron sequence in the mitochondrial intermembrane region. Plays an important role in regulating mitochondrial morphology and function by cleaving OPA1 at position S2, giving rise to a form of OPA1 that promotes maintenance of normal mitochondrial structure and mitochondrial protein metabolism. Ensures cell proliferation, maintains normal cristae morphology and complex I respiration activity, promotes antiapoptotic activity and protects mitochondria from the accumulation of oxidatively damaged membrane proteins. Required for normal, constitutive degradation of PRELID1. Catalyzes the degradation of OMA1 in response to membrane depolarization. Required to control the accumulation of nonassembled respiratory chain subunits (NDUFB6, OX4 and ND1).

Target Involvement

Optic atrophy 11 (OPA11)

Target Subcellular Location

Mitochondrion inner membrane. Mitochondrion.

Target Protein Families

AAA ATPase family; Peptidase M41 family

Target Tissue Specificity

High expression in cardiac and skeletal muscle mitochondria.

Target Synonyms

YME1L1; FTSH1; YME1L; UNQ1868/PRO4304; ATP-dependent zinc metalloprotease YME1L1; ATP-dependent metalloprotease FtsH1; Meg-4; Presenilin-associated metalloprotease; PAMP; YME1-like protein 1

Target Background

The protein encoded by this gene is the human ortholog of yeast mitochondrial AAA metalloprotease, Yme1p. It is localized in the mitochondria and can functionally complement a yme1 disruptant yeast strain. It is proposed that this gene plays a role in mitochondrial protein metabolism and could be involved in mitochondrial pathologies. Three transcript variants encoding different isoforms have been found for this gene.